Treatment of the Chronically Transfused Patient (2)

Transfusion requirements 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. Infusion rates average 7.5 ml of blood per kg/hr for non-cardiac patients and 3 cc/kg/hr for patients with cardiac failure. 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.

Iron Overload and Desferal Administration
Detailed annual iron assessments are determined by quarterly ferritin levels, liver biopsies and dose-dependent urinary iron studies which are performed selectively. Recently, SQUIDs have been used to assess hepatic iron storage in patients when liver biopsy is felt to be unacceptable. The results were compared to liver biopsy and ferritin levels to evaluate consistency of the three measurements of overload in our population. The results confirmed the value of using both SQUID and liver biopsy.

In brief, chelation is initiated when the transferrin approaches complete saturation; generally this is when the ferritin reaches 1000 ng/ml. As described elsewhere, this is preceded by a detailed educational program that includes Child Life, Social Services, Psychological and Home Care Services. Young children are started at reduced doses. Desferal is infused a minimum of 8-12 hours/day subcutaneously for a minimum of 5 days/week. Continuous intravenous infusion is used for selective patients. This group generally includes patients whose ferritin reaches 3000 ng/ml, and patients with organ failure. Desferal toxicity is monitored every six months via vision and hearing screening. Growth is monitored quarterly to check for Desferal-related growth delay. At each transfusion visit, the nurse practitioner, psychologist and pediatrician discuss Desferal use with the family. A record of Desferal dosage is maintained in the chart. Home Desferal and health care service is coordinated by the nurse practitioner. <next>

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