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Galactosemia is a genetic disorder that affects how the body processes a simple sugar called galactose.
It causes a buildup of galactose and its byproducts in the blood, which can lead to complications and health problems.
Galactose is found in some foods, especially milk and dairy products.
The most common treatment for galactosemia is a low-galactose diet.
This article explains what to eat and avoid on a low-galactose diet and provides a three-day sample low-galactose diet menu.
What is galactosemia?
Galactosemia is a genetic condition where the body lacks the enzymes necessary to process galactose (1).
Galactose is a simple sugar that, together with glucose, forms lactose.
Although galactosemia is sometimes confused with lactose intolerance, the two conditions are unrelated.
In lactose intolerance, the body doesn’t produce enough lactase, the enzyme needed to digest lactose, whereas in galactosemia, the enzymes that are needed to process galactose — not lactose — are severely deficient or missing completely.
There are three enzymes necessary for processing galactose:
- galactokinase (GALK)
- galactose-1-phosphate uridyltransferase (GALT)
- uridine diphosphate-galactose-4-epimerase (GALE)
A deficiency in any of these enzymes leads to high levels of blood levels of galactose and its byproducts, resulting in galactosemia.
There are four main types of galactosemia, depending on which enzyme is deficient (1):
- type 1, or GALT deficiency
- type 2, or GALK deficiency
- type 3, or GALE deficiency
- type 4, or galactose mutarotase (GALM) deficiency
Of these, type 1 or classic galactosemia is the most common and most severe form.
Galactosemia presents at birth and can cause signs such as feeding intolerances, vomiting, and poor weight gain.
Galactosemia is usually identified through state newborn screening programs, which measure the activity levels of the three enzymes necessary for processing galactose.
The most common treatment for galactosemia is a low-galactose diet (1).
There are different guidelines for following a low-galactose diet during infancy and from childhood to beyond.
A soy-based or elemental formula should be used to replace breast milk or standard infant formulas that contain lactose.
Lactose or milk sugar is made up of both glucose and galactose.
Eliminating lactose can be difficult since infants rely on breast milk or infant formula as their sole source of nutrition.
Fortunately, there are plenty of lactose- and galactose-free formulas available.
Recommended formulas for infants with galactosemia include (2):
Avoid ready-to-feed or liquid concentrate formulas because galactose-containing ingredients are commonly added to these liquid forms.
While a low-galactose diet is essential for preventing life-threatening health complications in newborns, especially when implemented in the first week of life, it may not prevent long-term complications from galactosemia, such as brain impairments, premature ovarian failure, and reduced bone health (1).
Childhood and beyond
Galactose restriction should be continued through childhood and beyond.
But its restriction becomes less important after infancy and early childhood when breast milk or formula are no longer the primary sources of nutrition (2).
Currently, there is no age-related recommendation for the amount of galactose allowed on a low-galactose diet (3).
Instead, current guidelines recommend a low-galactose diet that only eliminates sources of lactose and galactose from milk and dairy products (3).
Examples of dairy products to eliminate include:
- cow’s milk
- cream cheese
- cottage cheese
- ice cream
- whipped cream
You should also avoid products that use dairy as an ingredient, such as some meal replacement drinks or bars, dairy-based salad dressings, puddings, and custards.
You can read the ingredient list if you’re unsure whether a product may contain milk or lactose.
Look for ingredients like milk protein, milk sugar, malted milk, sour cream, and buttercream.
While similar sounding, lactate, lactic acid, and lactylate are not lactose and are acceptable ingredients.
Foods to eat with galactosemia
Beyond milk and dairy products, many other foods like honey, cherries, celery, and even some spices like paprika, basil, and onion powder also contain galactose (4).
However, guidelines don’t recommend restricting these foods since they contain galactose in small amounts, and doing so hasn’t been shown to offer significant benefits (3).
As such, all foods without lactose or galactose from dairy products can be included in your diet.
Here are foods to eat with galactosemia:
- Fruits: apples, apricots, bananas, berries, grapes, melons, oranges, peaches, pears, etc.
- Vegetables: arugula, asparagus, beets, broccoli, carrots, green beans, mushrooms, onions, peppers, spinach, squash, etc.
- Starches and grains: barley, bread, corn, oats, pasta, potatoes, rice
- Legumes: chickpeas, black beans, green peas, kidney beans, lentils, lima beans, peanuts
- Nuts and seeds: almonds, Brazil nuts, chia seeds, flax seeds, hazelnuts, pecans, pine nuts, pistachios, quinoa, walnuts
- Meats: beef, buffalo, goat, lamb, pork
- Poultry: eggs, chicken, duck, pheasant, turkey
- Seafood: cod, crab, herring, lobsters, mackerel, salmon, scallops, tuna
- Oils: canola and olive oil
- Beverages: coffee, plant-based milk alternatives, tea, water
Milk and dairy products tend to be good sources of calcium and vitamin D, but you can still get plenty of these nutrients with a varied diet.
Calcium and vitamin D are especially important for supporting good bone health since decreased bone mineral density is a common complication of galactosemia (5).
Good sources of calcium include (6):
- fortified orange juice
- fortified soymilk
- fortified breakfast cereals
Good sources of vitamin D include (7):
- cod liver oil
- fortified soy milk
- fortified breakfast cereals
Vitamin K is also good for bone health, with collards, spinach, kale, broccoli, soybeans, and pumpkin being among the best sources (8).
3-day sample low-galactose diet menu
Here’s a three-day sample low-galactose diet menu, which includes a vegan option:
- Breakfast: scrambled eggs and oats made with fortified soy milk
- Lunch: fish tacos
- Snack: apple slices and peanut butter
- Dinner: grilled salmon, roasted potatoes, and sauteed asparagus
Day 2 (vegan)
- Breakfast: tofu scramble and oats made with fortified soy milk
- Lunch: black bean tostadas
- Snack: chia pudding with slivered almonds and blueberries
- Dinner: Mediterranean chickpea salad
- Breakfast: fortified breakfast cereal with chopped nuts and a banana
- Lunch: green salad with bacon, avocado, and hard-boiled eggs
- Snack: orange slices and almonds
- Dinner: beef and broccoli with rice
The bottom line
Galactosemia is a genetic condition that prevents your body from properly processing galactose, a simple sugar and component of lactose.
Complete galactose restriction is necessary during infancy to decrease the risk of health complications but it becomes less important in early childhood and beyond.
After infancy, all foods with lactose or galactose from dairy products must still be restricted from the diet whereas other foods that contain small amounts of galactose can still be included.