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Is There a Special Diet for Thalassemia?

      Hemoglobin, the molecule in red blood cells that carries oxygen from the lungs to the body's tissues and returns carbon dioxide from the tissues back to the lungs, is made of two protein chains: alpha globin and beta globin. When genes controlling hemoglobin production are missing or altered, thalassemia occurs and is classified as alpha thalassemia or beta thalassemia. Hundreds of alpha globin and beta globin mutations have been identified in thalassemia, and the severity of the disease depends on which mutation or combination of mutations is present.

      National Heart Lung, and Blood Institute: Explore thalassemias. http://www.nhlbi.nih.gov/health/health-topics/topics/thalassemia/. Accessed May 27, 2016.

      Mild forms of the disease may not need any treatment, but those with moderate to severe thalassemia may be treated with life-sustaining blood transfusions. Regular blood transfusions greatly contribute to the quality and length of life of thalassemia patients, but leave patients with progressive iron deposition in body tissues that can result in organ injury due to iron overload. Despite receiving no or only occasional blood transfusions, patients with non–transfusion-dependent thalassemia can also develop iron overload due to increased intestinal absorption of dietary iron, which can accumulate iron to levels comparable with transfusion-dependent patients.
      • Taher A.T.
      • Viprakasit V.
      • Musallam K.M.
      • Cappellini M.D.
      Treating iron overload in patients with non-transfusion-dependent thalassemia.
      Iron overload can be successfully managed with chelation therapy to remove excess iron from the body.

      National Heart Lung, and Blood Institute: Explore thalassemias. http://www.nhlbi.nih.gov/health/health-topics/topics/thalassemia/. Accessed May 27, 2016.

      • Taher A.T.
      • Viprakasit V.
      • Musallam K.M.
      • Cappellini M.D.
      Treating iron overload in patients with non-transfusion-dependent thalassemia.
      Considering the relationship between iron overload and organ dysfunction in thalassemia, it is not surprising that dietary iron reduction has for decades been the focus of nutrition intervention in patients with thalassemia. However, the Standard of Care Guidelines for Thalassemia published by the Children’s Hospital & Research Center Oakland makes the following recommendations specific to iron:
      • Non-transfused patients are encouraged to consume a moderately low-iron diet—that is, avoiding iron-fortified foods and excessive consumption of red meat.
      • For transfused patients on chelation therapy, a low-iron diet is unnecessary and may decrease quality of life for some patients.

        Standards of Care Guidelines for Thalassemia. Northern California Comprehensive Thalassemia Center. UCSF Benioff Children's Hospital Oakland. http://thalassemia.com/treatment-guidelines-1.aspx#gsc.tab=0. Accessed May 27, 2016.

      In addition to iron-related issues, patients with thalassemia commonly exhibit morbidities linked with poor nutritional status: inadequate growth, poor immune function, and decreased bone mineralization. As part of the Thalassemia Clinical Research Network’s Longitudinal Cohort Study, research investigating dietary intake of thalassemia patients was published in the Journal in 2012.
      • Fung E.B.
      • Xu Y.
      • Trachtenberg F.
      • et al.
      Inadequate dietary intake in patients with thalassemia.
      Fung and colleagues studied patients with thalassemia across 10 hematology outpatient clinics in the United States and Canada, comparing their food frequency data with the US Dietary Reference Intakes and correlations with serum 25-OH vitamin D and total body iron stores. Results showed surprising levels of nutrient inadequacy in this population. Recommendations based on this preliminary data suggested that registered dietitian nutritionists shift the focus away from avoiding iron-rich diets and toward concentrating on a more well-balanced diet rich in antioxidants and minerals. When iron is avoided in the diet, zinc intake is frequently reduced; zinc is an essential nutrient that has been shown to be particularly beneficial to immune status, bone health, and growth in thalassemia. The intake of dairy foods is also low, which might, in part, be related to lactose intolerance. Therefore, strategies for increasing dietary calcium and magnesium should emphasize lactose-free dairy and calcium-fortified foods. Shifting the focus toward more fruits, vegetables, and whole grains will not only enhance antioxidant and fiber intake, but also enhance folate intake, which is critically important to red blood cell metabolism.
      In 2016, a study reviewed the relationship between nutritional status and three commonly experienced comorbidities in patients with transfusion-dependent thalassemia: low bone mass/osteoporosis, growth-deficiency and/or delayed puberty, and diabetes.
      • Fung E.B.
      The importance of nutrition for health in patients with transfusion-dependent thalassemia.
      The researcher concluded that on the whole, there are few well-designed, adequately powered studies on the role of general dietary or specific micronutrients in the cause, treatment, or prevention of these commonly observed morbidities in thalassemia. Until these data are gathered, it is suggested that patients with thalassemia be monitored frequently and that their nutritional deficiencies be corrected when observed in order to advance their overall health and quality of life. Until that time, optimizing dietary intake through nutrient-dense foods and appropriate use of supplements, where necessary, can improve overall health in these subjects.
      • Fung E.B.
      The importance of nutrition for health in patients with transfusion-dependent thalassemia.

      References

      1. National Heart Lung, and Blood Institute: Explore thalassemias. http://www.nhlbi.nih.gov/health/health-topics/topics/thalassemia/. Accessed May 27, 2016.

        • Taher A.T.
        • Viprakasit V.
        • Musallam K.M.
        • Cappellini M.D.
        Treating iron overload in patients with non-transfusion-dependent thalassemia.
        Am J Hematol. 2013; 88: 409-415
      2. Standards of Care Guidelines for Thalassemia. Northern California Comprehensive Thalassemia Center. UCSF Benioff Children's Hospital Oakland. http://thalassemia.com/treatment-guidelines-1.aspx#gsc.tab=0. Accessed May 27, 2016.

        • Fung E.B.
        • Xu Y.
        • Trachtenberg F.
        • et al.
        Inadequate dietary intake in patients with thalassemia.
        J Acad Nutr Diet. 2012; 112: 980-990
        • Fung E.B.
        The importance of nutrition for health in patients with transfusion-dependent thalassemia.
        Ann N Y Acad Sci. 2016; 1368: 40-48