SOURCES OF DIET GUIDELINES
Unfortunately, clinics do not provide uniform direction to their parents and patients, the recommended diet for Classic Galactosemia is somewhat controversial. Below is a list of diet resources that some parents follow, however, please always check with your own clinic for diet advice. We encourage families to gather as much information as they can, work with their clinic, so that they can make the best possible decision they can for their own child.
1) Galactosemia Diet Resources from University of Colorado Inherited Metabolic Diseases Nutrition Department
Books and educational materials are intended for Health Care Professionals and families of children with classical galactosemia. The following is a description of the books, which can be ordered directly from the University of Colorado through their order form. Download "Galactosemia Order Form" (PDF)
Galactosemia: The Diet
The purpose of this book is to review the sources of galactose in the diet, describe which foods are included in the diet, and provide tips for healthy eating while on a galactose-restricted diet.
Galactosemia: For New Parents
This book is intended for parents with infants who have been diagnosed with galactosemia due to a deficiency of the enzyme galactose-1-phosphate uridyltransferase or GALT. This book will provide an overview of the diagnosis and familiarize you with the diet for galactosemia.
Galactosemia: Making Healthy Choices Cookbook
This cookbook contains recipes and educational information about the galactosemia diet.
Galactosemia vs Lactose Intolerance
In Galactosemia, the body does not produce a functional Galactose-1-Phosphate-Uridylyltransferase enzyme thus the body is not able to fully metabolize galactose sugars. One method galactose is introduced to the body is through metabolization of lactose, a "complex" sugar that contains a galactose sugar and a glucose sugar. Excessive galactose in the body leads to a number of significant acute complications (see What is Galactosemia). Currently there is no therapy that permits a person with Galactosemia to eat an unrestricted diet, without concern of complications.
Lactose intolerance is an inability in the body to digest significant amounts of lactose. This inability is often the result of a shortage of Beta Galactosidase (Lactase) enzyme, which is normally produced by cells that line the small intestine. Lactase metabolizes lactose into galactose and glucose. When lactase is limited, a person with intolerance may feel distressed and have intestinal gas, bloating and diarrhea, though there typically no other acute or chronic complications. Some with lactose Intolerance, allow limited amounts of lactose in diet as they have some lactase activity and can safely do so without complication. Others use Lactaid and Dairy Ease (Lactase) enzymes that can be taken orally. These options are not available in Galactosemia. Thus while literature on lactose intolerance diets has similarities
WEBSITES OF INTEREST (GENERAL INFORMATION ON RESTRICTED DIETS)
Since actual products change on a regular basis it is imperative that you re-read every label every time you buy. Various parents and dietitians have put together this list of unacceptable ingredients in an attempt to simplify the ingredient dilemma.
Please, remember to always check with your clinic and dietitian. Gather all information available and then make your own decision.
Food Ingredients which are unacceptable in the diet for Galactosemia:
Cheese (EXCEPTIONS: Jarlsberg, Gruyere, Emmentaler, Swiss, Tilster, grated 100% Parmesan, Parmesan aged >10 months, and sharp Cheddar cheese aged >12 months)
Dry Milk Protein
Dry Milk Solids
MSG (Monosodium Glutamate)****
Nonfat Dry Milk
Nonfat Dry Milk Solids
Organ Meats (liver, heart, kidney, brains, pancreas)
Fermented Soy products and Soy Sauce*****
Whey and Whey Solids
NOTE: Lactate, Lactic acid and Lactylate do not contain lactose and are acceptable ingredients.
* Dough Conditioners may include caseinates which are UNACCEPTABLE. Most labels specify the name of the conditioner which is added to the product. If not, contact the company to make sure that all are acceptable.
** Hydrolyzed protein is UNACCEPTABLE and is commonly found in canned meats, like tuna. Hydrolyzed vegetable protein, however, is acceptable.
*** A few diet margarines do not contain milk. Check labels before using any brand. If "margarine" is listed as an ingredient in any processed food, consider the product UNACCEPTABLE.
**** MSG or Monosodium Glutamate itself is acceptable; however, some MSG's contain lactose extenders. It is best to avoid MSG whenever possible.
***** Soy sauce is UNACCEPTABLE if it is fermented. Brands must be checked before including this in the Galactosemic diet.
Given the necessary restriction on dairy items in the galactosemic diet, parents sometimes wonder whether their child with Galactosemia is getting sufficient calcium.
Ask your doctor or clinic what the recommended calcium intake is for your child. If your child sees a nutritionist, you may ask him/her to perform a three-day diet analysis to determine if your child is getting enough calcium (as well as other nutrients).
There are a number of natural food sources of calcium among the foods acceptable for a galactosemic diet. If you are advised to increase your child's daily intake of calcium, it may be best to try to increase these natural sources in your child's diet before turning to supplements.
Advice on calcium supplements varies from clinic to clinic. Below is a list of calcium supplements that some parents are using. Keep in mind that this list just represents some of the types of calcium supplements given to children with galactosemia and is NOT an endorsement for any of the products. As with anything, check labels carefully (for restricted ingredients) and always check with your own doctor/clinic before giving any supplement to your child. Keep in mind companies frequently change ingredients in their products.
Tums - some are still using, although there has been talk about Tums being bad for tooth enamel among other things
Centrum Vitamins - "Bone Health" - suppose to be lactose and dairy-free (It is like a regular vitamin)
CalQuick (Twin Labs) - liquid calcium supplement (600 mg/tablespoon)
Liquid Cal Mag+ (KAL) (600 mg/tablespoon)
Multi-vitamins with extra calcium
Keep in mind that there are different forms of calcium used in supplements (e.g. calcium carbonate, calcium phosphate, calcium citrate, calcium gluconate, etc.). Some forms of calcium are thought to be more easily absorbed into the body than others. Another factor to consider is that levels of calcium in the blood may not always indicate the actual calcium used by the body to increase bone density. Ask your nutritionist for advice on this matter.
Calcium recommendations vary depending on age and special needs. In addition, levels of estrogen can affect calcium needs in women. Note: Calcium requires adequate Vitamin D to be absorbed into the body
GALACTOSE CONTENTS IN FOOD
GALACTOSEMIA FOUNDATION RECIPE BOOK
We have put together a lot of great recipes. You can download our recipe book here.