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The severe forms of thalassemia are the most common inherited anemias managed with regular blood transfusion therapy. Transfusion policies and complications are critical to quality of life and survival, but there is a lack of standardized care.A survey of 58 items was completed in 2016 by 11 centers in California, Washington, Oregon, Nevada, and Arizona providing long-term care for thalassemia. The questionnaire addressed demographic information, transfusion practices and complications, and educational needs.The centers followed 717 patients with β-thalassemia (314, 43.8%) or α-thalassemia (394, 55%). One-third (34.7%) of patients were transfusion-dependent. Indications and goals of transfusion therapy differed between centers. Prestorage leukoreduction was universal, while routine irradiation of units was limited to one site. Red blood cell antigen phenotype was determined before the first transfusion and patients received Rh/Kell-matched units. However, more than half of the transfused patients had received blood at multiple hospitals within or outside the United States. Alloantibodies were seen in 16.9% of transfused group, but management of such patients was variable. Unusual or emerging transfusion-transmitted pathogens were not observed. Multiple educational needs were recognized, with iron overload as the biggest challenge; the approach to iron chelation varied within the group.This study identified many patients not included in earlier surveys limited to major national centers, suggesting that the thalassemia population in the United States is vastly underestimated. Lack of evidence-based guidelines is a barrier to optimal care, which should be addressed through regional consortia of thalassemia centers.

Chronic red blood cell (RBC) transfusion has been used for decades to prevent the classical bone changes and life-threatening anemia in patients with homozygous β-thalassemia (Thal).1 More recently, transfusion therapy has also been used to minimize disease-specific morbidity in patients with sickle cell disease (SCD).2,3 Long-term transfusion therapy without adequate chelation, however, results in iron overload–associated organ failure.4–7 Consensus documents have been developed for patients with Thal8–10 and adopted for use in SCD,11,12 to provide clinicians guidance in the monitoring and treatment of chronically transfused patients. Quantitative iron assessment by percutaneous liver biopsy, the most viable clinical option at the time of this study, was recommended to be performed every 2 years, in addition to quarterly assessment of serum ferritin. Details for annual monitoring of cardiac, endocrine, and hepatic function were also provided. In 2002, the National Institutes of Health (NIH) released a consensus document for the care of patients with SCD receiving transfusion therapy.13 The document summarized that “there is no evidence that SCD patients are spared the fatal consequences of iron overload. Therefore a comprehensive program to monitor and treat iron overload is necessary.” To date, there have been no studies directly comparing the adequacy of care provided in these two similarly transfused patient populations. Disparity in the delivery of health care has gained attention over the past decade.14–16 In its report of racial and ethnic disparities in health care, the Institute of Medicine (IOM) concluded that there were significant disparities in the quality and quantity of health care received by minority groups in the United States.17 In 1994, the Sickle Cell Treatment Act was passed designed to improve funding and resources for specialized SCD centers. Despite significant advances in the treatment of SCD, a 2006 report suggests that major funding gaps remain when support is compared between patients with SCD and cystic fibrosis, a chronic disease affecting far fewer Americans who are primarily Caucasian.18 This report highlights federal and philanthropic research support; however, the quality of clinical care provided is undoubtedly impacted. Although solutions remain elusive, it is clear that health care disparity is a complex issue that cannot be explained solely on the basis of research support, a patient’s race, socioeconomic status, access to health care, behavior, or physician knowledge or pre-paredness. The objective of this article is to evaluate the adequacy in the assessment of iron overload and management of iron-related organ toxicity in two similarly transfused but distinct patient groups.