A Teacher's Guide to Galactosemia

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Hi, my name is _________________________________

I have galactosemia and I want you to learn more about it so you can help me. This booklet will tell you about galactosemia and my special diet.


Galactosemia Basics

These are the facts...

Galactosemia is an inherited disorder of carbohydrate metabolism that affects the body's ability to utilize certain sugars from food. Galactose is a sugar that may be found alone in foods but is usually found as part of another sugar called lactose.  

circle with half colored in signifying lactose with an arrow pointing to a half circle signifying glucose followed by a plus sign and a half circle colored in signifying galactose

Lactose is sometimes called milk sugar and is made of equal parts of simple sugars galactose and glucose. The simple sugars can be absorbed directly into the bloodstream after digestion.

Children with galactosemia are missing the enzyme to convert galactose to glucose for energy in the body. If galactosemia is left untreated, galactose will accumulate in the blood and body tissues and will cause damage. A newborn with untreated galactosemia may develop vomiting and diarrhea and fail to gain weight. The build-up of galactose can eventually lead to jaundice, an enlarged liver, cataracts, intellectual disability, and possible death. 

The good part is...

Many states routinely check a newborn's blood during the first week of life to see if the infant has galactosemia. The infant with galactosemia can then be put on galactose-free formula to prevent any dietary galactose from entering the blood stream. The immediate response to dietary treatment is excellent and the effects of the disorder are greatly alleviated. Blood monitoring is then done on a regular basis. 

And now the diet...

To treat galactosemia all sources of galactose and lactose must be eliminated from the diet. That primarily means that all dairy products from any animal and foods with dairy products in them must be strictly avoided. Except for galactose, children with galactosemia need the same kinds and amounts of nutrients as any other children to grow and develop normally.

An infant with galactosemia is given a milk substitute, usually a soy formula. Solid foods that do not contain galactose are introduced in a timely sequence, as with any other infant. A child with galactosemia continues to drink a prescribed milk substitute and avoids foods that contain lactose. Except for the milk substitute, no special dietary products are needed.

galactosemia
food group of milk and cheese crossed out with red x food group of meat, fish, poultry food group of fruit vegetables

The child learns at an early age that his or her diet is restricted and to ask if a new food is allowed. By school age, children with galactosemia usually know a good deal about their own diets.

Parents have complete, detailed lists of foods to use and avoid in feeding their child with galactosemia. Some examples:

Detailed lists of foods
traffic light

NOT ALLOWED: All forms of milk, cheese, yogurt, cottage cheese, cream cheese, ice cream, sherbert, pudding, creamed soups and sauces, milk chocolate.

CAUTIOUSLY READ LABELS to look for milk products in bread, crackers, cookies, chips, cereal, pancakes, hot dogs, bologna, breaded meat and fish, gravy, processed foods, artificial sweeteners, non-dairy creamers, drugs.*

ALLOWED: Most other meats, eggs, legumes, fruits, vegetables, grains, breads, soy milk, fats, and sweets that do not contain galactose or lactose.

*It is very important to read the ingredient label before serving any processed or packaged foods or medicines to the child with galactosemia. Many foods and medicinal products have lactose added during processing even though they do not appear to contain any milk products. Manufacturers often change the ingredients, so labels should be reexamined at frequent intervals. When in doubt, consult the parents or choose a different product for the child.

labels
CHECK INGREDIENT LABELS IN PACKAGED FOODS

Safe Ingredients
Lactate, Lactic Acid, Lactalbumin

Unsafe Ingredients
Butter, Casein, Caseinates, Cheese, Cream, Curds
Lactose, Milk, Dry Milk Solids, Whey Whey Solids

 

The outlook is...

Medical researchers have begun to look at the long term consequences of galactosemia in groups of children. It is becoming increasingly apparent that the long term outcome with regards to intellectual development, performance, and behavior may not be as good as expected despite early diagnosis and continued dietary control. Despite IQ values in the normal range, children with galactosemia may have learning problems. Speech and language deficits, visual perceptual difficulties, and social adjustment problems have been documented. These results are based on averages from groups of children with galactosemia. An individual galactosemic child may not necessarily exhibit all or any of the noted problems. 

How you can help...

Treat the child with galactosemia as a strong, healthy member of the class. Be alert to special learning needs, but remember that none may exist in this particular child.

Keep a good line of communication open with the child's parents to reinforce parent teaching and monitoring of food eaten away from home. The child's parents are your closest experts on galactosemia.

Let the parent know

... if the child has eaten any food not allowed.

... if the child does not eat foods that are sent from home.

... if the child trades food with classmates.

birthday cake... if special occasions such as birthday or holiday parties are planned to make sure there is a galactose-free food for the child. Parents may want to send a supply of allowable treats that store well and can be available for special or unexpected occasions.

PLEASE do not feed the child with galactosemia ANY food not allowed on the previous list or approved by his/her parents. Even "little tastes" containing galactose add up and result in an elevation in their blood. While the child's curiosity may be aroused by these foods, he/she is accustomed to doing without them. 

Questions & Answers

How often are children with galactosemia born?

Approximately one newborn in every 50,000 births has galactosemia. This amounts to 60 to 100 new cases each year in the United states. There is more than one type of inborn enzyme defect that causes galactosemia and several variant forms have been recognized. The severity of the disorder and treatment may vary somewhat. The child's parents can provide you with additional details if you need them.

Since galactosemia is inherited, do all the children in that family have galactosemia?

figure showing parents with one normal and one galactosemia gene and four children one with normal genes one two with one normal and one galactosemia called carriers and one child with galactosemiaFor a child to have galactosemia, each of the parents must be a "carrier" of the galactosemia gene. A carrier has one normal gene and one galactosemia gene, but can metabolize galactose normally. A child with galactosemia inherited a galactosemia gene from each parent. When these parents have offspring, there is a 25 percent chance that the baby will be free of the galactosemia gene, a 50 percent chance that the baby will be a carrier, and a 25 percent chance the baby will have galactosemia. With each pregnancy, there is the same 25 percent chance that the newborn will have galactosemia. In some families, there may be only one child with galactosemia, while in other families, mulitiple children may be affected. Galactosemia affects both boys and girls.

Is galactosemia the same as lactose or milk intolerance?

dairy cow with a line through itNo, galactosemia should not be confused with lactose intolerance. People with galactosemia usually have no problems digesting lactose or absorbing galactose. The problems occur after galactose has entered the blood stream. People who are lactose intolerant must avoid large amounts of lactose in foods but can usually still digest and metabolize galactose. Commercial enzymes that are available to break down lactose in foods do not alter the amount of galactose in the food, and therefore are of no help to people with galactosemia. Strict avoidance of foods with lactose and galactose is the only known treatment for galactosemia.

If a child with galactosemia eats a food with galactose, will he or she feel sick?

If a child with galactosemia does eat a food with galactose in it, he or she may not feel sick or different in any way. However, some children with galactosemia are more sensitive than others and may experience discomfort. It is the elevated blood levels over time from the continued ingestion of galactose that leads to serious complications. The changes may not be seen for several weeks or months. Because of this slow and subtle effect, it sometimes makes it difficult for a child with galactosemia to understand that milk products and other foods are harmful.

Without dairy products, does the child with galactosemia grow like other children?

The child with galactosemia grows just like the other children in the classroom. The important protein, vitamins, and minerals that the other children get from dairy products are provided in the prescribed milk substitute that children with galactosemia drink. Without a milk substitute, the child with galactosemia needs to take a daily vitamin and mineral supplement.

How can I explain the galactosemia diet to the other children in the class?

Young children can understand that since cars with different engines use different fuel (gas, diesel, etc.), some children have bodies that work in different ways than others and they need different food. Older chiildren can understand the similar concept of a "food allergy."

Don't hide the fact that the child's dietary needs are different if asked, but no long explanation is needed. Speak with the child and/or parent privately to determine how best to answer this question.

When can the diet be discontinued?

Ask the child's parents. In the past, some children with galactosemia stayed on the diet only through infancy and early childhood. Most doctors today are advising families to continue with the diet indefinitely. The child with galactosemia will never be able to metabolize galactose in the normal way and will always be at risk for associated medical complications. 

Activities

This section has suggestions for classroom activities for all the children in your class. Be sure to check with the parents of a child with galactosemia in planning any food related activities. The parents can tell you for certain which foods are allowed. You might even get some extra help in the classroom that day.

The following pages present some ideas for activities which you can develop to meet the needs and abilities of your students.

school house

Differences are fun!

The child with galactosemia has a different diet from most other children. The teacher can use this situation to teach all the children that there are differences among people and it is these very differences that make the world more interesting.


"People are unique"

  • Make body tracings.
  • Collect photographs of each child and whole class.
  • Measure each child's height and weight.
  • Describe each other (verbally for the younger child, written for the older children).
  • Have each child take a turn showing the class how he/she is special.

chef "People eat different foods"

FOOD CUSTOMS:

  • Geography (seacoast, ranch lands, farms, etc.)
  • International (Mexican, Chinese, Italian, etc.)
  • Religious (Jewish, no pork; Hindu, no beef)
  • Seasonal (summer and winter fruits and vegetables)
  • Vegetarians (no foods from animal sources)

HEALTH:

  • Overweight (low calories and low fat)
  • High blood pressure (low salt)
  • Diabetes (controlled sugar)
  • Galactosemia (no dairy products)
  • Heart disease (low fat, low salt) 

Skill sharpeners!

Here are several activities which could strengthen developmental and academic skills needed by all children, but are especially important for the development of a galactosemic child's self-sufficiency. The following activities are appropriate for selective ages:

measuring cupMeasuring and Pouring Skills

Use cups and spoons to measure liquid and dry ingredients.



knivesFine Motor Skills

Use a knife to cut, chop and spread foods or playdough.




books

 

Academic skills

Follow a recipe
Keep food records
Read and collect food labels
Learn to budget money, calories, or time. 

Things to do with food!

Many nutrition activities can be adapted to use galactose-free foods so all children can participate in the same way. Remember to review the allowable foods with the cild's parents.

USE THE SAME FRUIT OR VEGETABLE 

  • Slice, chop, make juice
  • Prepare in different ways: raw, boiled, fried, broiled (corn can be on the cob, cutoff, popped, ground into flour)
  • Trace where a food comes from or go on field trips. Oranges are grown on a tree; squeezed or canned or bottled; sold in a store; taken home (or to school) to drink.

USE DIFFERENT FRUITS OR VEGETABLES 

  • Compare colors, shapes, textures, tastes
  • Show where grown (in ground, on a tree, bush, etc.)tree

Classroom baking projects can be adapted to include the child with galactosemia. Many recipes are available that utilize only galactose-free ingredients. Ask the child's family if they have any favorite recipes which they would share with you. 

Resources for activities

Creative Food Experiences for Children
Mary T. Goodwin and Gerry Pollen, 1974
Center for Science in the Public Interest
1755 S Street, NW
Washington, DC 20009

Very good for preschool and early elementary school classes with ideas for older children. It is often available in school libraries, school resource centers or public libraries.

Nutrition Super Stars
Arizona NET Program, June 1981
Developed by: Department of Nutrition and Food Science
University of Arizona
Tucson, AZ 85721

Distributed by: Nutrition Education and Training Program
Arizona Department of Education
1535 West Jefferson
Phoenix, AZ 85007

Developed for grades 5 and 6; the ideas can be adapted for younger children.

You, Me and Others
March of Dimes Birth Defects Foundation, 1985

Available from: Supply Division
1275 Mamaroneck Avenue
White Plains, NY 10605

or your local March of Dimes office

Learning programs about basic genetics for grades K-6.  

Developed by Maria Nardella, M.A., R.D. Office of Nutrition Services. Children's Rehabilitative Services. Arizona Department of Health Services. 1740 W. Adams Room 208. Phoenix, Arizona 85007. August 1986. (Partial revision Nov. 13, 2014.)

Last updated November 13, 2014