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Important information for Thalassemia Patients

Update on the SQUID Program
Dr. Zahra Pakbaz, MD, has been working with the thalassemia team at Children's Hospital & Research Center at Oakland in order to study different aspects of thalassemia, ranging from quality of life and adherence to treatment with Desferal to iron overload. Many patients with thalassemia intermedia are able to survive without routine blood transfusions and even with relatively low hemoglobin levels. It appears that using medications such as decitabine, hydroxyurea, and erythropoietin can help to increase their hemoglobin by 1 to 2 grams.

However, based on SQUID measurements of liver iron concentration in 26 non-transfused thalassemia intermedia patients, Dr. Pakbaz and the CHRCO team found that although they had relatively low serum ferritin levels, their liver iron was high, and they could become as iron overloaded as transfused thalassemia major patients. Iron overload of non-transfused thalassemics is primarily due to increased iron absorption from the intestine. Therefore, liver iron concentration needs to be monitored in patients with thalassemia intermedia, regardless of their serum ferritin, and it is also important that they consult with a dietitian to plan a low-iron diet. If iron overload goes untreated, it can cause serious complications.

For more information, please contact Dr. Pakbaz of CHRCO at zpakbaz@mail.cho.org or 510-428-3885, ext 4248

Update on Bone Densitometry (DXA)
Patients with thalassemia frequently have low bone mass, and we are finding that many adults experience significant bone pain and have increased risk for fracture. These complications are not limited to patients with thalassemia major, but are also observed in non-transfused intermedia patients, E beta Thalassemia and Hemoglobin H.

The most effective way to prevent low bone mass is to build strong, dense bones during youth. A combination of disease, endocrine issues, and nutritional factors likely contribute to the etiology of osteoporosis in patients with thalassemia. To help build healthy bones it is important to do the following:

  • consume a diet rich in calcium and vitamin D
  • participate in non-contact, weight-bearing physical activity as tolerated
  • get a measurement of your bone density every 1 to 2 years (DXA scan)
  • have an endocrine evaluation as indicated by your doctor
If you already have low bone mass, there are treatments that can improve and/or stop the condition from progressing. It is important to discuss this with your health care provider.

For more information or to schedule a DXA, please contact Ellen Fung, PhD, of CHRCO at efung@mail.cho.org or 510-428-3885, ext 4939.

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