Newborn Screening Case Management Galactosemia Handbook

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galactosemia handbook with a little bear image   This handbook is meant to give you the basic information that will help you understand how to care for your child with galactosemia.

Contact the Texas Department of State Health Services (DSHS), Newborn Screening Program at 1-800-252-8023 orEmail for more information.

In this handbook, "OK foods" have a heart poundingnext to them

and the "not OK foods" have a do not sign next to them.


What is Galactosemia? (ga-Lac-to-se-me-a)

Galactosemia is an inherited disorder that affects the way the body breaks down certain sugars. Specifically, it affects the way the sugar called galactose is broken down. Galactose can be found in food by itself. A larger sugar called lactose, sometimes called milk sugar, is broken down by the body into galactose and glucose. The body uses glucose for energy. Because of the lack of the enzyme (galactose-1-phosphate uridyl transferase) which helps the body break down the galactose, it then builds up and becomes toxic. In reaction to this build up of galactose the body makes some abnormal chemicals. The build up of galactose and the other chemicals can cause serious health problems like a swollen and inflamed liver, kidney failure, stunted physical and mental growth, and cataracts in the eyes. If the condition is not treated there is a 70% chance that the child could die.

Galactosemia is not the same thing as lactose intolerance. Galactosemia is more serious because it will cause permanent damage if left untreated.

How do you get Galactosemia?

Galactosemia is passed on from parents to their child. Technically it is called an autosomal recessive trait, which means that both of the parents of the child with galactosemia are carriers of the disorder. The carriers of the disorder will not get sick. But when two carriers have a child together there is a 1 in 4 chance (25%) the child will have galactosemia, a 2 in 4 chance (50%) the child will be a carrier of the disease, and a 1 in 4 chance (25%) the child will not be a carrier or have the disease. These are the chances with each birth.

 

chart displaying mother and father carriers of galactosemia would possibly have two children born as carriers, one child with galactosemia, and one that is neither a carrier nor has the disease

If one child in a family is diagnosed with Galactosemia then all children born before and/or after should also be tested. Each new baby in the family should be started on a galactose-and lactose free formula even before the enzyme test results are back. If you had planned to breast-feed your baby, pump your breast milk until the test results come back. If the new baby does not have galactosemia, the baby can be breast-fed or changed to a milk-based infant formula.

During pregnancy an amniocentesis test may detect if the infant has Galactosemia.

What problems can Galactosemia cause?

Studies show that over the years children and young adults with galactosemia can have problems with speech, language, hearing, fine-motor coordination, hemorrhage of the gel-like substance of the eye, tremors, stunted growth, and certain learning disabilities. Galactosemia is different for each child and will not cause the same problems with all children. Even when all instructions are followed, some children do not do as well as others. Studies tell us that children who were not given the strict diet have an increased risk of having one or more of the problems listed above.

For most girls with galactosemia, a problem called ovarian failure can happen. Ovarian failure means that the ovaries (where the eggs for having babies come from) either do not develop before she is born or they won't work as she grows up. Again, because it is different for each girl, the best way to deal with this problem is to talk with her doctor to find out the latest information.

What is the treatment for Galactosemia?

A very strict diet is essential. With a restricted diet the child with galactosemia may have less severe symptoms. There is no chemical or drug substitute that can be taken for the missing enzyme. The treatment for galactosemia is to restrict galactose and lactose from the diet for life. This means that foods that have galactose and lactose must be avoided. Since galactose is a part of lactose, or milk sugar, all milk and all foods that have milk in them must not be eaten. This is not just cow's milk, but any animal's milk including goat's milk and human breast milk. This includes dairy products like butter, cheese, and yogurt. Other foods that have small amounts of milk products in other forms

no cheese   no milk

such as whey, casein, and curds must also be eliminated. Also some foods known as "organ meats" such as brain, liver, thymus and pancreas (sometimes called sweetbreads) should not be eaten because they contain stored galactose.

Sounds simple, right?

Yes, it does sound simple; just avoid all milk and anything made with milk or milk products. Well, it's not that easy to follow. In fact it will take a big effort to make sure what your baby eats does not have galactose or lactose in it. It will take discipline on your part. It will mean that you have to change some of your habits when shopping at the grocery store. You will also have to change the way you cook your child's food. Ingredients are always changing and the treatment for galactosemia is unclear. It is best that you follow the advice of your baby's doctor and nutritionist.

Do not feel guilty that you are denying things to your child. The special diet is for the health of your child. Other than giving your child with galactosemia a special diet, it is important that you treat him or her the same as you would any other child. Do not try to "make-up" for the special diet by giving special favors or putting up with behavior that is not acceptable. Setting limits and discipline are very important to teach your child with galactosemia. As your child grows older you will have less control over the diet than you had during infancy. At some point the child will grow into an adult and become completely responsible for his or her own diet. He will need the knowledge and discipline to maintain the diet on his own.

Never give "just a taste" of a "Not OK" food. If they never taste it they will never miss it.

Help your Child Live with Galactosemia

It is important that you help your child learn to eat safely, since he will live with Galactosemia all of his life. Teach OK and Not OK foods early while you teach colors, shapes, and other safety ideas like stop signs, looking both ways when you cross the street and holding a grown up's hand when walking in a parking lot. By the time your child starts kindergarten he should be able to tell people he has Galactosemia and milk and foods made with milk are not good for him.

Children love to help fix food for snacks, and can help plan meals when they get older. Ask your nutritionist to help you with activities for your child, so that by the 6th grade, your child can make safe independent decisions about what he eats each day.

Make dealing with galactosemia a natural part of life.

How will I know if a food contains galactose or lactose?

By reading labels. It is very important that you read the labels to see what ingredients are in foods. Make sure you read the label every time because ingredients are always changing. Many foods contain milk or some form of milk product either naturally or added during processing. If a food label has one of the following key words, it contains galactose or lactose and must be avoided:

  • Nonfat dry milk solids
  • Casein
  • Sodium caseinate
  • Whey
  • Whey solids
  • Curds
  • Lactose
  • Galactose
do not symbol

Some examples of foods that contain galactose or lactose are:

 

  • Creamed, buttered, or breaded vegetables
  • Dehydrated and canned soups
  • Most commercial cakes, cookies, candies and puddings.
do not symbol

The Following Food Ingredients are galactose free and are safe to use:

  • Lactate
  • Lactic acid
  • Lactalbumin
  • Stearoyl-2-lactylate
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Kosher foods are foods eaten by people who follow the Jewish dietary laws. The word "Parve" or "Pareve" will be on the label to indicate the food was manufactured free of animal and dairy products. Orthodox Jews may not have meat and milk products at the same meal. Parve products may be served at a Kosher dairy meal or a Kosher meat meal. However, no Parve product is allowed to have lactose or caseinates. You still must read the label to make sure it is free of caseinates and other galactose or lactose ingredients.

You also need to know that certain foods do not require a listing of ingredients, like candy for example. Also, food companies might use one term to describe ingredients such as "dough conditioner" and will not list the complete ingredients. Things like caramel coloring may or may not have galactose in them depending on how it was made. It is up to you to ensure that what you feed your child with galactosemia is free of galactose or lactose. If you have any question in your mind or are unsure of the actual ingredients, you should contact the manufacturer and ask if the food contains any galactose or lactose. Most food companies will answer your questions if you let them know why you are asking. The food company's address or website can usually be found somewhere on the label.

READ THE LABEL EVERY TIME, even if it is a product you have used before and know is galactose and lactose-free. Ingredients change even if the appearance of the label looks the same.

Another great place to get some advice on what is "OK" or "Not OK" for your child with galactosemia is the Internet. There are several organizations out there that can be helpful. Using an internet search engine such as www.altavista.com, www.hotbot.com or www.excite.com, just type in the key word "galactosemia" and you will be on your way. If information on the Internet contradicts information you have received from your child's physician and/or nutritionist, do not change treatment without discussing it with the physician.

Baby Formulas (Milk Substitutes)

To insure your baby will get the nutrients needed to grow, you will need to use some type of milk substitute. Soybean protein isolate formulas are suggested. They look the same as regular milk but smell and taste differently. Most babies will drink the formulas, especially if it is given to them at a very early age. New brands of formulas are always coming out; right now the formulas being used are:

  • Isomil® (Ross Laboratories)
  • ProSobee® (Mead Johnson Laboratories)
  • Isomil DR® (Ross Laboratories)
  • Alsoy® (Carnation)
  • Follow-up Soy® (Carnation)
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If for some reason your baby cannot drink the soy-based formulas, there are casein-hydrolysate formulas that have only a very tiny amount of galactose. They are:

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  • Nutramigen® (Mead Johnson Laboratories)
  • Pregestimil® (Mead Johnson Laboratories)
  • Alimentum® (Ross Laboratories)

While on the casein-hydrolysate formulas, the child must be checked every now and then to make sure that the galactose is not building up in the blood.

Do not use the following baby formulas; they are not good for babies with galactosemia:

do not sign
  • Enfamil® (Mead Johnson) whey, nonfat milk
  • Similac® (Ross) whey, nonfat milk
  • Good Start® (Carnation) whey
  • Lactofree® (Mead Johnson) milk protein isolate
  • Similac Lactose Free® (Ross) milk protein isolate
  • Follow-up® (Carnation) nonfat milk
  • Similac Neosure® (Ross) whey, nonfat milk
  • Enfamil 22® (Mead Johnson) whey, nonfat milk

Always check with the nutritionist at the metabolic center before making any changes in your baby's diet.

Soy formulas cost about the same as regular formulas. They are available in three forms:

  • Concentrated liquid (you add water)
  • Powder (you add water)
  • Ready-to-feed (no water added) costs more.

Be sure to follow the directions on the label when mixing the formula.

Milk Substitutes from Toddler to Adult

Serve the soybean protein isolate formulas in a training cup. Since the soybean protein isolate formulas are a good substitute for milk, it can be used to replace milk for the rest of your child's life. If the child is not drinking enough of the formula to meet their nutrition needs, then it might be necessary to give them Vitamin D and calcium tablets. Calcium and Vitamin D help your baby's bones and teeth grow strong and healthy. Ask the child's nutritionist or doctor what the right amount of formula is for your child.

An adult with galactosemia can continue to drink the soybean protein isolate formulas. There are also some beverages made for adults that have milk intolerance that are OK to drink. They may be found in the health food or dairy section. As always, read the label to make sure it is OK to use. These adult milk substitutes should not be used for children or infants because they don't have the full amount of vitamins and calcium. Regular milk that has added lactase to help people with milk intolerance digest it, should not be a part of the galactosemic diet even as an adult. Avoid milk substitutes that are made with whole soybeans, organic soybeans, soymilk, soy flour, and soy protein (unless soy protein isolate is specified). Brand names are always changing but here are some examples of what to avoid:

  • Lactaid® (added lactase)
  • Dairy Ease® (added lactase)
  • Coffee Mate® (caseinate)
  • West Soy® (whole soy bean)
  • Soy Dream® (whole soy bean)
  • Edensoy® (whole soy bean)
  • Mocha Mix® (soy protein)
do not sign

Milk substitutes that are made with isolated soy protein and some that are made from rice are acceptable. Some of the brand names are:

  • Vitamite® (isolated soy protein)
  • Rice Dream® (brown rice)
  • Pacific Rice® (brown rice)
  • Coffee Rich®
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Again, read the label to be sure they are OK to use and check with your nutritionist if you have any doubt.

Breads, Pastas, and Cereals

breadSome bread foods have milk added in some form. Read the labels carefully to make sure they don't. If you are not sure, you can write the manufacturer or bakery that makes the food and ask for the ingredients. They are usually happy to tell you if you explain why you are asking.

Pastas and Rice are usually OK to use. Things like:

  • Spaghetti
  • Macaroni
  • Noodles
  • Rice
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Most cooked and dry cereals are lactose free but if they have lactose it is usually in the form of whey. Again, ingredients are always changing so reading labels every time is important.

Plain saltines and graham crackers usually don't have galactose, but check the labels just in case.

Meats and Protein Foods

Plain meats are great sources of protein. Meats like:

  • poultry (chicken and turkey)
  • fish
  • eggs
  • beef
  • pork
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Meats that must be avoided are:

  • Liver
  • Thymus, pancreas, brain (sweetbreads)
  • Other meats with fillers
  • Creamed or breaded meats
do not sign

Fats

Fats that are OK:

  • pure oils
  • shortenings
  • lard
  • kosher margarines that are galactose-free
  • "diet" margarines (read the label)
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Fats that are not OK:

  • margarine
  • butter
  • prepared salad dressings (especially creamy ones)
do not sign

Fruits and Vegetables

cornucopiaFruits and vegetables contain very complex components that contain some galactose. Some of these components will release a small amount of galactose during digestion. Experts do not agree on whether or not these small amounts are a problem for someone with galactosemia. Ask your nutritionist; she will help you decide on what is best for your child.



The following fruits and vegetables are still questionable:

Fruits
Apple
Banana
Dates
Figs
Grapes
Kiwi
Pears
Watermelon
think bubble Vegetables
Broccoli
Brussel Sprouts
Carrots
Onions
Squash
Tomatoes
Sweet potatoes

 

Legumes (bean foods)
Peas
Lima beans
String beans
Dried peas
Pinto & kidney beans
think bubble

Soups & Sauces

soup canMany of the commercially canned soups, sauces, and gravies contain margarine. Read the label to ensure they are galactose or lactose free. If you prepare sauces and gravies at home, use water instead of milk.

Sweets

There is no need to cut out sweets from the galactosemic diet. Store bought sweets that are milk-free or homemade sweets using the OK ingredients are allowed.

Some good desserts or snacks are:

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  • Gelatin
  • Certain fruit (ask the nutritionist)
  • Fruit ices (with certain fruits)
  • Angle food cake
  • Fruit-flavored puddings made with water
  • Homemade sweets using O.K. ingredients
  • Unsweetened chocolate, carob powder, or cocoa powder
  • Any non-chocolate candy (read the label)
  • Gelatin-based candies like gumdrops, orange slices and Fun Fruits.
  • Hard candies like lemon drops & Life Savers
  • Sugar substitutes, saccharin and nutra sweet are allowed.

no ice cream Sweets made with milk products that should be avoided are:

  • Puddings
  • Custards
  • Ice cream & Ice milk
  • Sherbet
  • Yogurt
  • Anything with milk chocolate
do not sign

Prescription and Over-the-counter Medicines

bottle of medicine A lot of medicines have lactose added. Usually this is done to make the medicine taste better so kids will take it. They are called inactive ingredients. Sometimes these inactive ingredients are used as the "glue" that holds the pill in shape. Whatever the reason for using them, inactive ingredients are not always listed on labels. Check with your doctor or the pharmacist; they will look up all the ingredients in the medicine in a book called the "Physicians' Desk Reference." "PDR" for short. Most physicians and pharmacists have two PDR's, one copy for prescription medicine and another copy for non-prescription over-the-counter medicine.

Always ask the doctor if the medicine they are prescribing your child is free of galactose and lactose. If it isn't, then a similar medicine made by another manufacturer should be prescribed.

When buying over-the-counter medicines at the drug store, be sure to ask the pharmacist to check the PDR and see if the medicine has galactose or lactose. Be sure to tell the pharmacist why you are asking; they should be more than happy to help you. If the medicine does contain galactose or lactose, then ask the pharmacist to help you find another drug that can be used.

If for some reason your child must receive medicine by I.V. (intravenous), tell the medical personnel that your child has galactosemia and that medicines with galactose or lactose should not be used.

Fast Food and Eating Out

Eating out is not easy for anyone on a special diet. But, it can be done. It will take some effort and planning. Many restaurants will prepare special items. Ask for plain vegetables or rice instead of those cooked in butter or smothered in cream sauce. Ask if the burgers are made from pure ground beef. Take your own special galactose or lactose-free buns. Many fast food places have salads or salad bars so why not take your own special dressing? How about the potato bar (make sure to skip the sour cream, cheese and butter of course). A potato topped with real bacon bits and spices would be allowed. Take along the soy protein isolate formulas in ready-to-drink cans or a measured out amount of powder to be mixed with a glass of water at the restaurant.

Some foods to avoid:

  • Hot dogs
  • Processed meats
  • French fries
  • Anything breaded or creamed
  • Most buns
  • Creamy dressings
do not sign

It will take some time to get used to always asking for special items or questioning the ingredients, but in time it will become second nature to you. You might want to think about becoming a "regular" at certain restaurants; you will become familiar with the menu, and the staff will get to know you too and can help you make sure the food for your child is galactose and lactose free. Remember to always ask about ingredients or how the food was prepared even if you do become a "regular."

Going Shopping

Going to the grocery store is going to take more time than it did before, especially the first few times that you go. If you can make a few plans before going it might make it easier. Try these following tips:

  1. Shop at off times. By shopping when the store isn't so full it should reduce the amount of frustration you would feel if the store were crowded.
  2. Plan shopping days. Buy enough of each product to last a set number of days, for example enough formula to last a little over two weeks. Set a schedule and buy enough to last that amount of time to reduce the number of trips to the store.
  3. Shop in pairs. If you have someone that can go with you, two sets of eyes reading labels is faster than one. For couples, it will create a feeling that they are both in this together and that they both need to help watch the baby's diet.
  4. Leave the baby at home if you can. If someone can watch the baby for you, great. Any parent will tell you that they can shop faster and are more relaxed when they don't have their kids with them. It isn't always possible, but try.
  5. Make a list of products that are OK. After going shopping a few times you will have identified some brands or products that are OK to use. This list will help you shop a little faster but remember to always read the label.
  6. Buy in Bulk. When you can, buy concentrated or powdered formulas or other products in bulk. Many warehouse or pantry stores have some great deals when you buy in bulk.
  7. Buy dairy free staples for the whole family. Things like milk-free margarine or bread can be bought for the entire family to use. This makes it easier than trying to keep up with which bread is which.
  8. Make a list of things needed for the meals you have planned. Nothing is more frustrating when cooking than to find out you are missing one key ingredient. If you plan the meals and then make a list, you won't forget anything.
  9. Make up a 3"x5" Recipe Box and take it with you. Get one of those boxes that 3"x5" cards fit in and keep recipes that do not use milk or milk products in there. If you take this with you, you will be sure to get what you need and maybe even plan a meal you had forgotten about. You can also hold your coupons and other lists in this box.

School, Babysitters, Friends and Family

At first it won't be too difficult to control your child's diet. The diet is pretty simple for infants: the special formula and some galactose-free and lactose-free baby foods. When your child is an infant is a good time to start planning the diet and reading labels. It is also a good time to start practicing the "OK" ways to cook food for your child once they start weaning off the bottle. It is also important to start telling friends and family members about the baby's special diet. If the baby is in daycare or with a babysitter, it is very important to talk with the caretaker and explain galactosemia and the special diet.

When your child reaches toddler age and is in preschool, hopefully he will know to drink his special milk. The sooner your child accepts the diet as being "normal" the better off he will be. Educate the staff at the preschool about the special diet of the child so they can help too. You may need to supply special snacks for your child or arrange special meals. It is important to include your child in planning the diet. The older your child becomes the less you should do and the more responsible he should become for his diet.

Each year you should speak with your child's teacher and lunch room supervisor before the school year starts. If school lunches are not satisfactory then lunch at home or packing a lunch is necessary.

Resource Groups

Remember, you are not alone. There are other families out there that have children with galactosemia. Many of those families belong to groups and help each other by being pen pals, sharing recipes, and keeping up on the latest information about galactosemia. Contact the Texas Department of State Health Services Newborn Screening Program at 1-800-252-8023 for more information.

References

Parents of Galactosemic Children, Inc. (Fall 1998 Newsletter vol.10, Issue 1. PGCI 1741-I 2148 Bryton Dr., Powell, OH 43065

Parents of Galactosemic Children, Inc. "Food" On-line: www.galactosemia.org

Roberts, R. & Meyer, B. (1993) Living with galactosemia a handbook for families (3rd ed.), Indianapolis: Department of Pediatrics Indiana University School of Medicine, 702 Barnhill Dr., Indianapolis, Indiana 46202-5225

Please note: External links to other sites are intended to be informational and do not have the endorsement of the Texas Department of State Health Services. These sites may also not be accessible to persons with disabilities.

External email links are provided to you as a courtesy. Please be advised that you are not emailing the Texas Department of State Health Services (DSHS) and DSHS policies do not apply should you choose to correspond.

Newborn Screening Galactosemia Email

Last Updated April 22, 2005

Last updated January 07, 2011