Galactosemia Diet – 3 Important Tips

What is Galactosemia Diet

Lactose is a sugar found in dairy products, such as milk. When it is digested, it turns into galactose.

Galactosemia is a condition that happens when too much galactose builds up in the body.

This can cause damage to the liver, brain, eyes, kidneys, central nervous system, and other body systems. People with this condition need to follow a galactose-controlled diet for their entire life in order to avoid health problems.

Butter had previously been tested and although the amount of lactose is low, it is still considered too high for galactosaemia. Therefore butter is not allowed.

• Buttermilk
• Buttermilk Solids
• Cheese (EXCEPTIONS: Jarlsberg, Gruyere, Emmentaler, Swiss, Tilster, grated 100% Parmesan, Parmesan aged >10 months, and sharp Cheddar cheese aged >12 months)
• Cream
• Dry Milk
• Dry Milk Protein
• Dry Milk Solids
• Hydrolyzed Whey**
• Ice Cream
• Lactalbumin
• Lactose
• Lactoglobulin
• Lactostearin
• Margarine***
• Milk
• Milk Chocolate
• Milk Solids
• Milk Derivatives
• Nonfat Milk
• Nonfat Dry Milk
• Nonfat Dry Milk Solids
• Sherbert
• Sour Cream
• Whey and Whey Solids
• Yogurt

What are tips for following this Galactosemia Diet plan?

Reading food labels

• Always read ingredients lists carefully.
• Avoid foods that have ingredients such as butter, cream, milk, milk solids, lactose, casein, or whey. Ask your dietitian for a complete list of ingredients to avoid.
• Avoid foods with the label “may contain milk.”
• Avoid foods that contain monosodium glutamate (MSG). Some MSGs contain galactose, so it is best to avoid this ingredient.

Cooking

• Cook with vegetable oil, olive oil, grapeseed oil, peanut oil, or other plant-based oils. Do not use butter or margarine.
• Thicken soups or sauces using nondairy thickeners, such as flour, corn starch, or tomato paste. Do not use cream.

Meal planning

• Do not drink milk.
• Do not eat dairy products or foods made with milk-based products.
• Do not eat lactose-free foods, nondairy foods, or products that have been treated to have lactose removed.
• Avoid mixed foods, like pizza, casseroles, prepacked meals, or soups, unless you know they are made without milk or milk products.
• Get calcium in your diet from other nondairy foods, such as:
  • Sardines, canned salmon, and shellfish.
  • Tofu with added calcium (fortified tofu) and dried beans.
  • Kale, collard greens, turnip greens, and broccoli.
  • Calcium-fortified orange juice.

General instructions

• Work with your health care provider or dietitian to decide how restrictive your diet needs to be.
• Ask your health care provider or dietitian about taking a vitamin or mineral supplement. Some people on this diet do not get enough calcium, riboflavin, or vitamin D. Take vitamins and supplements only as directed.
• Do not take over-the-counter lactose digestive aids. These do not work for people with galactosemia.

What foods are allowed – Galactosemia Diet?

The items listed may not be a complete list. Talk with your dietitian about what dietary choices are best for you.

Some of these foods may contain small amounts of galactose. Talk with your health care provider or dietitian about which foods you can have and how much of them you can have.

Dairy

Soy, almond, rice, oat, or hemp milk. Soy yogurt, cheese, or sour cream. Almond or cashew cheese.

Meats and other protein foods

Any meat, poultry, and fish prepared without milk ingredients. Eggs. Nuts and seeds. Beans. Tofu.

Grains

Bread, cereal, rice, noodles, crackers, and baked goods made without milk ingredients.

Vegetables

All fresh, frozen, or canned vegetables prepared without milk ingredients. 100% vegetable juices.

Fruits

All fresh, frozen, or canned fruits prepared without milk ingredients. 100% fruit juices.

Fats and Oils

Plant-based oils. Sauces, salad dressings, and mayonnaise made without milk or milk ingredients.

Beverages

Black coffee and tea. Soft drinks. Coconut milk. Hypoallergenic commercial infant and toddler formulas.

Sweets and desserts

Sorbet, gelatin, or fruit popsicles. Soy, coconut, or rice milk ice cream.

Seasonings and other foods

All spices and herbs.

What foods are not allowed?

The items listed may not be a complete list. Talk with your dietitian about what dietary choices are best for you.

Dairy

All types of milk, including buttermilk, whole, reduced-fat, low-fat, fat-free, powdered, lactose-free, condensed, and evaporated milk. Cheese and cheese flavor. Cream, sour cream, and whipping cream. Yogurt.

Meats and other protein foods

Organ meat. Any meat prepared with a milk ingredient. Whey-containing protein supplements. Fermented tofu or soybeans.

Grains

Any baked good or grain dish made with a milk ingredient.

Vegetables

Vegetables prepared with milk ingredients.

Fruits

Fruits prepared with milk ingredients.

Fats and oils

Butter, butter fat, or butter solids. Margarine. Salad dressings made with milk ingredients. Cream cheese.

Sweets and desserts

Ice cream. Sherbert. Pudding. Milk chocolate.

Seasonings and other foods

Milk-based seasoning mixes. Ready-made sauce or gravy. Fermented soy sauce. Processed foods. Cream-based soups or sauces.

Beverages

Milk or yogurt-containing smoothies. Blended or premade coffee drinks.

Lactose Free foods

Lactose free or lactofree foods are dairy foods which are designed for people with lactose intolerance. Some lactose is removed and the remainder is enzymatically broken down into glucose and galactose. These products are still high in galactose and not suitable for people with galactosaemia. 

Food Ingredients which are unacceptable in the diet for Galactosemia: 

• Butter –

In a diet for Galactosaemia milk products have to be avoided due to the lactose content. There are several exceptions. The first is suitable cheeses which has been tested and found to be very low in galactose.

Mason and Turner were the first to report the successful use of a milk-free diet in an infant with galactosemia. In the 1950s, different strategies to feed infants a diet without galactose were discussed in the literature (Salt et al. 1955; Jones and Leak 1959).

Nowadays, initiating the diet is much easier, as infant formulas with a very limited amount of galactose are widely available. Still, there are dilemmas in the treatment of galactosemia, and the dietary treatment varies widely around the world. 

Back in 2014 the GSG tested butter oil and ghee to see if these are suitable in the diet for galactosaemia. They tested several batches and they all came out very low in galactose / lactose.

NOTE: Lactate, Lactic acid and Lactylate do not contain lactose and are acceptable ingredients.

Summary

• Galactosemia is a condition that happens when galactose cannot be digested by the body. People with galactosemia need to follow a galactose-controlled diet for their entire life in order to avoid health problems.
• This Galactosemia Diet plan involves avoiding foods and drinks that have milk, dairy products, and milk-based ingredients. Depending on your condition, you may need to avoid other foods as well.
• Ask your health care provider or dietitian about a detailed list of ingredients to avoid and whether you should take a vitamin or mineral supplement.

Affected newborns are recognized in the first days of their life, and special diet is introduced immediately.

The therapy of galactosemia is the lactose-free and galactose-poor diet for life.

As a result of the nationwide newborn screening and the lifelong medical therapy, early treatment with galactosemia can achieve a normal life without serious complications

Summary

Galactosemia is a rare genetic disorder in which the body is unable to break down galactose, a type of sugar found in milk and dairy products. As a result, people with galactosemia must follow a strict galactose-free diet to avoid the buildup of galactose in their bodies, which can lead to serious health problems. The galactosemia diet typically involves the following restrictions:

1. Avoidance of Milk and Dairy Products: All sources of milk and dairy products must be eliminated from the diet. This includes cow’s milk, goat’s milk, cheese, yogurt, butter, and any foods containing milk or milk-derived ingredients.
2. Lactose-Free Formulas: Infants with galactosemia cannot be fed regular infant formula, as it contains lactose from cow’s milk. Instead, they need to be given a specialized lactose-free formula that does not contain galactose.
3. Galactose-Free Foods: Foods that contain galactose or lactose as an ingredient must be avoided. This includes certain bread, cereals, baked goods, processed foods, and some medications that contain lactose.
4. Read Labels: People with galactosemia should carefully read food labels to check for hidden sources of galactose or lactose in packaged and processed foods.
5. Safe Foods: Foods that are safe to consume on a galactosemia diet include fruits, vegetables, meats, fish, eggs, and some grains, as long as they do not contain galactose or lactose.

Sources

Classic galactosemia