Nutrition and Osteogenesis Imperfecta

Good nutrition is important for all people with osteogenesis imperfecta (OI). Because OI is a genetic disorder of collagen, and not a calcium or other nutrient deficiency, there are no foods or supplements that will cure OI or prevent its symptoms. There has been little definitive research exploring the role of traditional or nontraditional supplements or specialized diets in OI treatment. Nevertheless, it is clear that to maintain optimal health, children and adults with OI should eat a balanced diet, which includes a variety of vitamins and minerals, and is low in fat and added sugar. Excessive weight gain should be avoided. Portion size, meal frequency, and total caloric intake may need to be adjusted depending on the severity of OI, body size, and activity level. Dental health may also affect what kind of foods a person with OI can eat, and how they are prepared.

Guidelines for a Healthy Diet: The Food Guide Pyramid

The U.S. Department of Agriculture has developed a Food Guide Pyramid to help people make healthy daily food choices. The pyramid illustrates six food groups, offering guidelines for the appropriate daily number of servings from each group. This pyramid was developed for people of average body size and activity level; people with OI may benefit from an individualized assessment from a physician or dietitian to determine the appropriate number of daily servings for their body size and activity level.

Keep in mind that the USDA's definition of a "serving" is usually much less than the average person's definition of a serving. For example, the USDA defines a serving of pasta as ? cup, whereas many people commonly serve themselves 2 cups or more of pasta, equal to four or more USDA servings of carbohydrates.

Eat 6 to 11 servings daily from the bread, cereal, rice, and pasta group. This group provides carbohydrates-a major source of energy-as well as fiber, vitamins, and minerals. A serving in this group is 1 slice of bread, 1 ounce of dry cereal, or ? cup of rice or pasta. To increase fiber in your diet, choose breads, cereal, and other starches made with whole-grain wheat, oats or bran. Many people believe that bread and pasta are "fattening" and should be avoided. Actually, these essential foods supply reasonable numbers of calories on their own. The calories skyrocket, however, if you add fats (e.g., cream sauce on pasta, butter on bread). Large portions also add on calories.

Eat 3 to 5 servings of vegetables, and 2 to 4 servings of fruit each day. High in fiber and nutrients, and naturally low in fat, these foods provide vitamins and minerals, such as Vitamins A and C, iron, folate, potassium, and some calcium. A serving is 1 cup of raw leafy vegetables, ? cup of chopped vegetables (raw or cooked) or fruit (raw, cooked or canned), 1 medium-sized piece of fruit, or ? cup of fruit or vegetable juice. In general, deeply or brightly colored vegetables and fruits pack the most nutrients. These include dark green leafy vegetables (spinach, kale, romaine lettuce), broccoli, carrots, sweet red peppers, winter squash (such as butternut), tomatoes, cantaloupe, strawberries, citrus fruits, kiwi, papaya, or mango. Many "juice drinks" have very little juice and a lot of sugar and water. Choose fruit juices that are 100 percent juice. Limit even these juices if weight is a problem (they can be high-calorie), and choose fresh fruit for fiber and to satisfy hunger between meals.

Eat 2 to 3 servings a day of milk, yogurt, and cheese, which provide protein and calcium. Adequate calcium helps develop and maintain bone mass. A serving from this group is 1 cup of milk or yogurt, 1? ounces of natural cheese, or 2 ounces of processed cheese. Choose low-fat or fat-free (skim) milk for drinking and in recipes. (Children under two years, however, need whole milk for proper development.) Choose reduced-fat (or "light") cheeses, yogurt, and sour cream over their higher fat counterparts when possible. (See the section on "Nutrition and Bone Health" for more information on calcium intake.)

Eat 2 to 3 servings from the meat, poultry, fish, dry beans, eggs, and nuts group. This group provides protein, B vitamins, iron, zinc, and other nutrients. A serving from this group is 2 to 3 ounces of cooked lean meat, poultry or fish. A ? cup of cooked dry beans (pinto, black, navy, etc.) or 1 egg is equivalent to 1 ounce of lean meat, and 2 tablespoons of peanut butter or 1/3 cup of nuts count as 1 ounce of meat. Choose lean cuts of meat-extra-lean ground beef, ground turkey, loin or sirloin cuts of beef, chicken or turkey breast, etc. Trim visible fat from meats before cooking, and remove the skin from poultry. Consume nuts and nut butters in moderation; they are nutritious, but high in fat.

Finally, use fats, oils, and sweets sparingly. Fat is necessary for energy and development, so work toward moderate fat intake, not a no-fat diet. Children under two especially need fat for their development-choose whole milk and avoid fat-free products for infants and toddlers. As much as possible, avoid buttering your bread, cooking and frying in oil, and adding fatty creams and sauces to foods. Sweets and desserts can be part of a healthy, balanced diet. Low-fat cookie and cake recipes usually substitute a fruit puree (such as applesauce), or low- or nonfat cream cheese, yogurt, or sour cream for some of the butter and shortening in traditional recipes. Remember, however, that low-fat does not equal low-calorie; limit the portion size and frequency of low-fat treats.

Nutrition and Bone Health

Calcium does not improve the basic collagen defects that cause OI, but people with OI need to get adequate calcium in their diets to develop bone mass and prevent bone loss, which can worsen bone fragility. In 1997, the National Academy of Sciences developed the following Recommended Dietary Intake for calcium:

Young children (1-3): 500 mg a day
Older children (4-8): 800 mg a day
Preteens/adolescents (9-18): 1,300 mg a day
Men and women (19-50): 1,000 mg a day
Men and women (50+): 1,200 mg a day
Pregnant/lactating women (under 18): 1,300 mg a day
Pregnant/lactating women (19-50): 1,000 mg a day

These guidelines were developed for people of average height and weight. A person with OI may have lower calcium needs. It is recommended that people with OI, particularly if they are much smaller than average, talk to a physician and/or registered dietitian about their individual calcium needs. A measurement of calcium in a 24-hour urine collection may help determine if a person with OI is getting adequate calcium.

An 8-ounce glass of milk (whole, low-fat, or skim), 1 ? ounces of cheddar cheese, 2 cups of cottage cheese, and 1 cup of yogurt each contain approximately 300 milligrams of calcium. In addition to dairy products, some calcium is also found in broccoli, kale, salmon (with bones), sardines (with bones), and some dried beans and nuts. Manufacturers are increasingly fortifying foods, such as orange juice, cereal, and breads, with calcium. A third-cup of almonds has 120 milligrams of calcium, a half-cup of cooked kale has 90 milligrams, and 8 ounces of fortified orange juice has 350 milligrams. Soy or rice milk enriched with calcium is another alternative for people who cannot tolerate cow's milk.

Soy-based foods can also supply some calcium, and some researchers believe that these foods contain other substances (phytoestrogens) that may boost bone health. Tofu is a solid form of soy, usually found in packaged blocks in the produce section of your supermarket. (Choose tofu with calcium sulfate listed in the ingredients.) "Silken" tofu puréed in a blender or food processor, and mixed with herbs, garlic, beans, or vegetables, makes for creamy dips and pasta sauces. It can also be used as a custard-like base for pies or fruit shakes.

If you are unable to get the appropriate amount of calcium for your body size and age solely through your diet, a calcium supplement may be appropriate. Check with your doctor before taking a supplement; he or she may want to check your calcium status through a urine collection before making a recommendation. Calcium supplements are sometimes recommended for people taking certain medications; check with your physician to determine if this is necessary.

Vitamin D is necessary for calcium absorption. Exposure to sunlight usually allows your body to manufacture as much Vitamin D as you need. Ten to 15 minutes of sun exposure a day is adequate. Vitamin D is also available in supplements and in some fortified foods. The Recommended Daily Allowance of Vitamin D is 400 IU (international units). Taking supplements with more than the RDA of Vitamin D can be harmful.
Vitamin C has many functions in the body, including the production of healthy connective tissues, and the healing of wounds and fractures. Vitamin C is abundant in many fruits (such as citrus fruits, strawberries, and cantaloupe) and vegetables (including tomato, bell peppers, and sweet potato). It is fairly easy to get adequate Vitamin C through the diet. There is some evidence that Vitamin C tablets can increase the risk of kidney stones in people who already have high levels of calcium in the urine. Because high urine calcium affects some people with OI, check with a physician before taking Vitamin C supplements.

Weight Control

Extra weight can impede mobility, put additional stress on weak bones, and increase the risk of other health problems. The best way for children and adults with OI to maintain a healthy weight is to get most of their calories from low-fat, high-nutrient foods, and exercise to the greatest extent safely possible. Consuming at least five fruits and vegetables each day is essential.

One way to control weight while eating a wide variety of foods is to control portion size. In a nation where huge portions-such as giant muffins, half-pound hamburgers, and 32-ounce sodas-are widely available, many Americans have mistaken perceptions of what constitutes a reasonable portion. Though it is not necessary to measure food at every meal, try doing it once or twice, so you have a picture in your mind of a reasonable serving of meat, pasta, cereal, etc. The American Dietetic Association offers guidelines to help you be more conscious of portion size:

* A ? cup of pasta is equal to an ice cream scoop. A ? cup of rice is equal to a cupcake wrapper.

* Three ounces of meat is the size of a cassette tape or a deck of cards. Three ounces of fish is the size of a checkbook.

* One ounce of cheese is the size of four dice.

* A medium potato is the size of a computer mouse.

* An average bagel is the size of a hockey puck. Half of an average bagel equals one serving of bread.

When mobility is limited, people with OI may gain weight, even if they are consuming a reasonable number of calories for body size, and eating a balanced diet. Talk to your doctor or physical therapist about ways you (or your child with OI) might burn calories through safe exercise. The recommended calorie intake for average-sized teens and adults ranges from 1,900 per day for women over 50, to 3,000 per day for teenaged boys. However, these levels are based on people of average height and weight, without mobility limitations. People with OI may benefit from consultation with a registered dietitian or nutritionist about appropriate calorie intake. Always talk with a physician or registered dietitian before beginning a very low-calorie diet, which may deprive you of necessary nutrients.

Constipation

Constipation is a problem for some people with OI. A high-fiber diet, drinking plenty of water and other fluids (particularly for those prone to excessive perspiration), and physical activity may help remedy this problem.

Tips for Adults

* Eat a variety of foods. A varied diet is the best way to ensure that you get all the nutrients you need. It also keeps you from getting "bored" and tempted to splurge on high-fat treats.

* Look for resources to help you modify your diet. For people raised in the "meat and potatoes" tradition, it can be intimidating to cook new foods, such as meatless entrées or unfamiliar fruits and vegetables. Invest in a basic low-fat cookbook, or a food magazine that focuses on healthy eating. A number of food and nutrition web sites also offer low-fat recipes.

* If you consume alcohol or caffeine, do so in moderation. Some experts believe alcohol and caffeine may be detrimental to bone health. Substituting milk, water, or 100 percent fruit juice for alcohol or caffeinated beverages will improve your overall nutrient intake.

* Make a grocery list. Before you shop, select several healthy recipes you plan to use in the coming week, and include the ingredients on your list. Having a list and a basic meal plan will help you avoid impulse purchases of processed junk food-and will probably save money too.

* Buy healthy "convenience" foods. Precut vegetables from the salad bar, prewashed salad greens in plastic bags, packaged roasted chicken breast, shredded reduced-fat cheese, and other convenience foods can cut down on preparation time and make healthy meals easy.

* Don't skip meals. Do allow yourself healthy snacks between meals. If you're ravenous by the time dinner comes, you're more likely to overeat.

Tips for Parents

* Avoid using food as a reward, or withholding food as a punishment. This sets up an unhealthy relationship between your child and food, which can contribute to overweight or eating disorders.

* At mealtime, fill everyone's plate with moderate portions directly from the stove or oven, rather than putting the food on the table "family style." Assure the kids that they can have seconds if they're still hungry after eating their portion.

* Serve milk, water, and 100 percent fruit juices with meals and snacks. Reserve sodas for special occasions. Your children may be more likely to consume healthy beverages if they see you doing the same.

* Keep healthy foods available for snacking. Single-serving containers of applesauce or pudding, carrot sticks with a low-fat dip, air-popped or low-fat microwave popcorn, and banana or apple slices with a little peanut butter are good alternatives to chips and cookies.

Web Sites for More Information

There are many web sites with nutrition information and recipes to help you eat healthier. Here are a few. If you don't have online access, many of these organizations publish brochures, magazines, and books that are available through your local library or bookstore.

American Dietetic Association www.eatright.org
This site includes hundreds of daily nutrition tips, nutrition fact sheets, and information on finding a registered dietitian in your area.

Betty Crocker www.bettycrocker.com
The Betty Crocker site includes a weekly menu planning feature, which provides a week's worth of recipes, along with a grocery list. Menus are organized by category (healthy, quick and easy, kids, etc.)

Cooking Light Magazine www.cookinglight.com
This site includes selected recipes from Cooking Light magazine, as well as daily tips and menu suggestions.

Five-a-Day Site www.dcpc.nci.nih.gov/5aday/
This site promotes the National Cancer Institute's "Five a Day" program to encourage Americans to eat more fruits and vegetables. It includes recipes.

Food and Nutrition Information Center www.nalusda.gov/fnic/index.html
The U.S. Department of Agriculture, which publishes the Food Guide Pyramid, offers this resource to explain the Pyramid and other nutrition guidelines. It includes links to the National Academy of Sciences, which publishes the Recommended Daily Allowance (RDA) of calories, vitamins, and minerals.

Healthfinder www.healthfinder.gov
Healthfinder is a comprehensive federal government web site with links to hundreds of health-related web sites, including nutrition sites.

National Osteoporosis Foundation www.nof.org
The National Osteoporosis Foundation's web site includes detailed information on calcium and its relationship to bone health.



This information is brought to you by the
NIH Osteoporosis and Related Bone Diseases~National Resource Center (ORBD~NRC)
and the Osteogenesis Imperfecta Foundation

National Institutes of Health
Osteoporosis and Related Bone Diseases
National Resource Center
1232 22nd St., NW
Washington, DC 20037-1292
Tel: 800/624-BONE or 202/223-0344
Fax: 202/293-2356, TYY: 202/466-4315
http://www.osteo.org
E-mail: orbdnrc@nof.org

The National Resource Center is supported by the National Institute of Arthritis and Musculoskeletal and Skin Diseases with contributions from the National Institute of Child Health and Human Development, National Institute of Dental and Craniofacial Research, National Institute of Environmental Health Sciences, NIH Office of Research on Women's Health, Office of Women's Health, PHS, and the National Institute on Aging. The Resource Center is operated by the National Osteoporosis Foundation, in collaboration with the Paget Foundation and the Osteogenesis Imperfecta Foundation.