Treatment of individuals with non-tranfusion dependent Thalassemia (2)

Nutrition 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.

Orthopedic Thalassemia intermedia patients are particularly at risk for orthopedic abnormalities, including craniofacial changes, fracture, osteoporosis, and spinal cord compression. At each visit, these patients are evaluated for signs and symptoms of hyperplastic marrow with bone density studies and endocrine evaluations, if necessary. Severe craniofacial abnormalities or spinal cord compression require emergency treatment followed by chronic transfusion therapy. Adequate calcium intake is encouraged.

Psychosocial The thalassemia intermedia patient can experience psychological problems such as decreased self-esteem, concerns of psychosexual development, and expectations of the future. These are intensified in patients who have language and educational barriers secondary to immigration. All adolescents are seen biannually by a genetic counselor, social worker, and psychologist to address these issues. Included in these group sessions are discussions of reproductive health and peer relations. This is done with a sensitivity to the patient's cultural beliefs as well as their religious and ethnic background.

Chemotherapy Treatment should include an offer of chemotherapy for increasing fetal hemoglobin with hydroxyurea or Butyrate therapy. This approach has been successful in selected individuals. The doses of hydroxyurea are much lower than those used to treat sickle cell disease, and toxicity must be carefully monitored. <back>

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