Below is an excerpt from these guidelines about nutrition. We can add more to this, but these are the basics and what should be tested is listed.
15 Nutrition
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.
Standards of Care Guidelines for Thalassemia • 25
Notes:
All trace elements (zinc, copper, selenium) need to be collected
into trace element–free vacutainers.
Normative values may be somewhat different depending
upon the reference lab. The upper limit for vitamin D is
10,000 IU when taken daily; much higher doses (e.g.,
200,000 IU) have been used in vitamin D–deficient
patients when taken weekly or monthly.
1 mg vitamin E = 0.45 to 0.67 IU vitamin D, depending
upon the form of vitamin E.