Galactosemia and the Dairy-Free Diet

Galactosemia is a rare metabolic disorder that affects how the body processes galactose, one of the simple sugars created when lactose is broken down in the digestive system. Not to be confused with lactose intolerance, classic galactosemia is a much more serious medical disorder. It does require a dairy-free diet. I’ll explain why and provide resources for living with this condition, and its variants.

What is Galactosemia?

During digestion of dairy, lactose (a complex milk sugar) is broken down into two simple sugars, glucose and galactose. The galactose and glucose then enter the bloodstream where the galactose is converted in more glucose. Glucose is used as a primary source of energy for the body.

People with galactosemia may have no problem digesting lactose. But they have a shortage or absence of the liver enzyme GALT, which is needed to break down galactose into glucose. As a result, galactose accumulates in the body, where it may damage the liver, central nervous system, eyes, kidneys, and other body systems.

What is the Prevalence of Galactosemia?

Classic galactosemia is the more severe form, where there is a missing or inactive GALT enzyme. It presents at infancy, and even affects the child’s ability to nurse. This is a very rare condition that is diagnosed in about 80 newborns in the United States each year. It affects about 1 in 30,000 to 1 in 60,000 infants.

There are other types of galactosemia, which can be more common. Some can be as severe as the classic type, and some don’t even present with symptoms. Duarte galactosemia is the most common type, and is diagnosed in 1 in 4000 babies. It is a milder form that may or may not require treatment.

What are the Symptoms of Galactosemia?

As mentioned, classic galactosemia presents itself quite soon after birth, with symptoms of vomiting, diarrhea, jaundice, and failure to thrive. Diagnosis is usually made during the first week of life with a heel prick blood test as part of standard newborn screening. Since galactose is a lactose by-product, babies with the more severe forms of galactosemia will not be able to consume any lactose-containing foods, including their own mother milk.

Some other types of galactosemia, like Duarte, might not present any symptoms. If there are no symptoms, there is usually some GALT enzyme working.

What is the Treatment for Galactosemia?

Treatment for classic galactosemia and some other variants requires the strict lifetime exclusion of lactose/galactose from the diet. This includes all milk products, but it can also include processed foods, some fruits and vegetables, meats, and some children’s medicines. Even with a prompt diagnosis and treatment, galactosemic children can have long-term complications.

In milder cases of galactosemia, where there is some functioning enzyme, treatment might be temporary or not needed at all. This will be addressed by the doctor at diagnosis.

Is a Dairy-Free Diet Required for Galactosemia?

With classic galactosemia, a strict dairy-free diet is typically required. This includes all forms of dairy-based and dairy-derived ingredients, and all types of mammal dairy. Unlike milk proteins, lactose, and consequently galactose, are the same in all mammalian species.

In milder forms of galactosemia, a temporary dairy-free diet might be recommended, or no treatment might be needed at all. This will be addressed by the doctor at diagnosis.

Are Lactose-Free Products Okay for Galactosemia?

Unfortunately, lactose-free products aren’t suitable for galactosemia patients that require treatment. To make lactose-free products, the manufacturer adds an enzyme to break the lactose down into glucose and galactose. This makes it easier to digest for people with lactose intolerance. But the lactose-free product is still filled with galactose, which is the problem for people with galactosemia.

Living Well with Galactosemia

We’ve received stories over the years from parents of children with galactosemia. Here are two, the first with the classic type, and the second received a more severe diagnosis at first, but it was later diagnosed as the Duarte type.

A Classic Case

I have a healthy, 12-year old daughter who was born with Galactosemia which is a metabolic disorder that does not allow the body to break down galactose – a milk protein. She has been on a dairy-free diet since she was 9 days old. At that time, I began my search to find any and every commercial product that did not contain dairy or dairy derivatives such as whey, lactic acid, sodium caseinate, etc.

Fortunately, we also keep kosher and the kosher “pareve” labeling system that appears on a package is a way to assure that the product does not contain dairy or dairy derivatives. It was very surprising to learn that things such as a simple, brand-name can of tuna can contain dairy! It has also been my goal to find a way to adapt recipes to make them dairy free so that my daughter could eat the “same” things as her friends.

I know that when someone’s child is first diagnosed, it feels like a death sentence because of all of the possible problems that can be caused by Galactosemia. My daughter’s first year was a tough one, but now the dairy-free diet is a no-brainer and she is a happy, healthy 12-year old. ~ Jessica

Discovered it was Duarte

Almost 4 years ago I found your site when my 2 month old son was diagnosed with Galactosemia. My son is almost 4 now and doing wonderful. After further testing, it was discovered that he had the variante case, Duarte Galactosemia, meaning that he could not have milk/milk products for the first year of his life, but then could slowly be introduced to milk/milk products.

I am writing you again because we are about to have baby number two. I am not stressed about how I will deal should this child have any version of Galactosemia because I have this website to turn to. Now I know that I will be able to find everything I need to make any adjustments I may need to make. I just wanted to say thank you for still being here. ~ Jennifer

Galactosemia & Dairy-Free Resources

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Galactosemia is a Dairy-Free Diagnosis, Not Just Lactose-Free