Nutrition and Diet

Nutritional deficiencies are common in thalassemia, due to hemolytic anemia, increased nutritional requirements, and morbidities such as iron overload, diabetes, and chelator use.

Patients should be evaluated annually by a registered dietitian regarding adequate dietary intake of calcium, vitamin D, folate, trace minerals (copper, zinc, and selenium) and antioxidant vitamins (E and C). Annual nutritional laboratory testing should include albumin, 25-hydroxy vitamin D, fasting glucose, fasting plasma zinc, serum copper, ceruloplasmin, serum selenium, alpha and gamma tocopherol, plasma ascorbate, and serum folate. (See nutrition table below.)

Recommendations for dietary supplementation should be made as indicated by nutritional history, complications of the disease, and, in children, growth status. Typically multivitamin supplementation without iron is suggested (e.g., Centrum Silver in tablet or chewable form is now available).

For nontransfused thalassemia patients, folate supplementation (1 mg daily) is recommended, and consuming a moderately low-iron diet is encouraged—that is, avoiding iron-fortified cereals and other products and excessive consumption of red meat. Drinking black tea with meals is recommended to reduce iron absorption from food.

For transfused patients on chelation therapy, a low-iron diet is unnecessary and may decrease the quality of life for some patients. The amount of iron obtained from just one unit of packed red cells (200 mg) far outweighs the amount of iron obtained from a 3-ounce steak (5 mg).

Vitamin D supplementation (50,000 IU once a week until levels normalize) is recommended for patients with a 25-hydroxy vitamin D less than 20 ng/dL. Calcium supplementation should be encouraged if dietary intake is insufficient.

Counseling should be offered for patients with special dietary needs. These include patients with diabetes or lactose intolerance, those who practice vegetarianism, those who are pregnant, or those on oral chelators or bisphosphonate medications.

Alcohol consumption and cigarette smoking are to be discouraged. Alcohol potentiates the oxidative damage of iron and aggravates the effect of hepatitis B and C on liver tissue. Cigarette smoking affects bone remodeling and is associated with osteoporosis.

Diet for the Non-Transfused Patient

Non-transfused thalassemia intermedia patients are encouraged to avoid high-iron and iron-supplemented foods, and encouraged to drink tea with meals, which decreases iron absorption. Serum ferritin is evaluated in adolescents. Desferrioxamine is instituted early in the development of hemosiderosis. Iron overloaded individuals receive a liver biopsy. Early cardiac evaluation with Holter monitoring and stress ECHO cardiogram is done in individuals with significant hemosiderosis. In addition, folic acid deficiency appears to be more common in these individuals.

Low Iron Diet for the Chronically-Transfused Patient

Regular blood transfusions can lead to iron overload in the body. Extra iron from chronic transfusions is stored in the liver. Once the liver stores are full, the iron begins to accumulate in places like the heart and pituitary, where it can do damage. Iron overload can also result from increased absorption of iron from the gut, as can be the case with thalassemia intermedia.

To help keep the iron stores from building up too fast, a medication called Desferal will be used in conjunction with a low iron diet. Keep the iron under 10 mg/day for those children under 10 years old, and under 18 mg/day for those who are 11 years old and older.

Children who have thalassemia and are transfused are still relatively anemic, so their bodies might still crave iron. As it may be difficult to watch their diets closely, they should develop good habits early. Remind children to definitely avoid very high iron foods such as dried beef and other high iron beef products, even if they are craving it. Remember that the iron found in meat is much more easliy absorbed than other sources of iron, such as cereals and breads.

Do not cook with cast iron cookware (e.g. a wok) because iron from the cookware can transfer onto the food. Some foods, such as orange juice, can enhance iron absorption, while others, like tea, dairy and coffee, can decrease absorption. If you are using Desferal, however, it is recommended that you take 250 mg or less of vitamin C after beginning infusion to help increase output of iron.

On food labels, the percentage of iron in one serving of that food is usually listed. This is based on the U.S. Recommended Daily Allowance of 18 mg/day. If the label says the food contains 8% of the daily recommended iron, multiply .08 by 18mg to get the mg iron from a serving of that food. Don't forget to check candy bars and snacks!

Foods to Avoid

Very high iron sources are found in the following foods; they should be avoided or eliminated from the diet:

Proteins that should be avoided or eliminated from the diet:

• oysters
• liver
• pork
• beans
• beef
• peanut butter
• tofu
• Grains that should be avoided or eliminated from the diet:
• flour tortillas
• infant cereal
• cream of wheat
• Malt-O-Meal
• cereals, such as Most, Product 19, Total, Kix, All Bran, Life, Raisin Bran, Special K, 100% Bran, Rice Chex, Rice Krispies, Cornflakes, Wheaties
• Fruits/Vegetables that should be avoided or eliminated from the diet:
• prune juice
• prunes
• watermelon
• spinach
• leafy green vegetables
• dates
• raisins
• broccoli
• peas
• Fava beans

Nutrition for Transfused Patients

Calcium and Thalassemia

Calcium is an essential mineral for building and maintaining strong bones and teeth. Calcium is found in some vegetables such as broccoli and kale, but dairy products such as milk, yogurt, and cheese are much better dietary sources of calcium. Some people either do not like dairy or cannot tolerate a dairy product in their digestive system. For these individuals a good source of calcium can be either calcium fortified orange juice or Tums, an antacid made solely of calcium carbonate.

A diet with inadequate calcium will decrease the storage of calcium in bones, which can become weak and will fracture easily. Weakness of bones is called osteoporosis. The calcium stored in your bones must last you your whole life, so if you have weak bones when you are a young adult it will be difficult to increase their strength as you get older. The peak time for storage is during the teenage years. It is during this time that the bones reach their adult length and strength. It is also during this time that estrogen in young women and testosterone in young men help them develop their bones and make them strong. If there is not enough calcium in their diets, or if there is not enough hormone production, the amount of calcium that is made into bone will be reduced. Since this is the major period of bone development, the bones will be weaker for the rest of a person's life.

Having strong bones is important for all of us, but it is especially important for people with thalassemia. Some of the secondary health problems that occur in children and adolescents with thalassemia affect bone formation. If thalassemia major is not treated with proper blood transfusions, there is so much activity in the bone marrow that bones will become thin and will fracture spontaneously. With adequate transfusion this is not a problem, but with transfusions come other problems; namely, iron overload. If an inadequate amount of Desferal is used and iron builds up in the body, the iron deposition can affect some of the organs that help the body build up strong bones. The major problem that interferes with strong bones is hypogonadism. Iron deposits in the testis in young men and in the ovaries in young women. This iron deposition can cause early menopause in young women in their twenties and can require hormone replacement therapy in both men and women. Without estrogen and testosterone, bones will not form normally and later in life the bones will be susceptible to disease. With replacement of estrogen or testosterone and adequate amounts of calcium, this problem can be prevented. Other problems of calcium metabolism can occur when the thyroid gland is affected by iron overload, and particularly when the parathyroid is damaged by iron.

What can be done to prevent problems related to bone strength and fractures?

The major problems can be delayed or prevented by the adequate use of Desferal to prevent damage to the ovaries and testes by iron. In addition, everyone, including children and young adults with either transfusion dependent or non-transfusion dependent thalassemia, should have an adequate intake of calcium, either by eating foods high in calcium or by taking calcium supplements. Exercise in moderate amounts also increases bone strength. It is hard to determine how much calcium is in your diet without knowing how much calcium is contained in foods. At an annual comprehensive clinic visit, each patient can visit a nutritionist to help him or her determine whether enough calcium is coming from the diet. Most people need about 1.5 grams of calcium daily to build strong bones. Children need less: about 1 gram per day. Adolescents need more during this time of increased bone growth: 1.5 to 2 grams per day. Adults need about 1.5 grams per day. These levels of calcium can be achieved by eating foods high in calcium:

High calcium foods

(one serving = 400 mg or .400 g of calcium)

1. One cup of milk

2. yogurt

3. milk based pudding or custard

4. 6 sardines with bones

Medium calcium foods

one serving = 125 mg or .125 g of calcium)

5. One cup tofu

6. One cup of beans* or peas

7. One cup broccoli, kale, mustard greens*

8. One half cup of bok choy or turnip greens*

9. One half cup cottage cheese, frozen yogurt, cream soup, or ice cream

10. One quarter cup almonds

11. 2 ounces canned fish with bones

Remember that if you are on a low-iron diet, try to find alternate sources of calcium, as these tend to be high in iron as well.

Many foods are fortified with calcium: orange juice, breads, soy milk. Look on the labels for both the iron content (avoid iron) and the calcium content of prepared foods. Also important is vitamin C for calcium absorption. If you think about calcium and how important it is for your health, you can easily increase the calcium you receive everyday. If you want more information about your diet, call your health care provider or a nutritionist for a consultation.