Input from each provider is solicited at case conference following the comprehensive clinic and used to construct a care plan. Transfused patients also undergo two semi-annual visits each year to monitor overall status. Care for the transfused thalassemia patient focuses on the most frequent complications including transfusion, iron overload and Desferal administration, endocrine failure, cardiac complications, splenomegaly, infectious disease, hepatitis, and psychosocial complications. Our approach to each of these problems is briefly described below. Pre-transfusion hemoglobin is maintained between 9.5 and 10.5 to ensure a mean hemoglobin of 12.
All patients receive leukodepleted phenotypically matched units.
Washed cells are used if urticarial reactions occur. Detailed clinical transfusion record for each visit includes grams of blood transfused, pre and post transfusion hemoglobin, code number of each unit, annual red cell consumption, and mean annual hemoglobin. Detailed annual iron assessments are determined by quarterly ferritin levels, liver biopsies and dose-dependent urinary iron studies which are performed selectively. The results were compared to liver biopsy and ferritin levels to evaluate consistency of the three measurements of overload in our population. As described elsewhere, this is preceded by a detailed educational program that includes Child Life, Social Services, Psychological and Home Care Services.