Construction of An Outpatient Erythrocytapheresis Program for Pediatric Patients with Sickle Cell Disease

An Erythrocytapheresis program was started at our institution in October 2006 for pediatric patients with sickle cell anemia who suffered from stroke(n=4) or chronic pain syndromes(n=3) with iron overload (n=6). Singer et al. (1999) along with others demonstrated that erythrocytapheresis is useful in preventing iron overload, and decreasing blood viscosity. In spite of the benefits there are many barriers to starting such a program, including repeated intravenous access, cost, location and complications of central venous devices. Five patients received double lumen Titanium 9.6 gauge Vortex pheresis port. Temporary double lumen, femoral pheresis catheters were placed and removed for each procedure in 2 patients exclusively and in a third patient who initially had a port. Non-coring Pheresis 16 gauge high flow needles were used to access ports manufactured initially by Arrow and now BAXA. Immediately after accessing, heparin (1000units/ml) was removed and Altepase (2 mg/ml) was placed in each lumen for 30 minutes. The Carolinas Region American Red Cross performed the pheresis procedure by Cobe Spectra following manufacturer guidelines. Once the procedure was completed each lumen of the port-a-cath was flushed with 1000 units/ml of heparin to fill the volume of the lumens. Ionized calcium levels were monitored and calcium gluconate was given as needed for each procedure. The goal was to keep the patient hemoglobin at 10 g/dl and %sickle hemoglobin less than 30% except for 2 patients in which we set a target percentage of sickle hemoglobin less than 20%. One patient had progressive stenosis and one had a repeat stroke on chronic erythrocytapheresis. These were the only two patients that had progressive disease and these were the only patients who had episodes of % sickle hemoglobin between 31% and 46% (two occasions for each patient). Average time of pheresis was 1 hour and 40 minutes. Initial pheresis procedures were performed in the pediatric intensive care unit (90 procedures) and those with ports were performed in an outpatient setting without increase in complications (20 procedures). All Patients were typed and crossed 2 days before procedure for phenotypically matched blood. The initiation of the pheresis program required cooperation from the American Red Cross, inpatient and outpatient nursing, PICU, radiology, blood bank, laboratory and the practice business managers. The providers and nursing staff developed a standard policy for pheresis port management and an order set for the scheduled monthly procedures. Secure emails between the hematology providers, the Red Cross, hospital blood bank, and nursing staff would confirm scheduled dates, times and the amount of blood needed for each procedure. North Carolina Medicaid usually reimbursed 25% of the pheresis procedure charges which did not include blood products, supplies and physician fees. Two patients with ports have had significant complications:

1. SVC syndrome and

2. Infection associated with surgical placement resulting in port removal.

One patient using the femoral pheresis catheter developed atrial flutter during the procedure. Serum ferritin for all patients decreased. None of the patients developed red cell allo-antibodies. All patients’ using femoral pheresis catheters did not have any clinical signs or radiographic abnormalities suggestive of thrombus or scarring. Vortex ports proved reliable in outpatient erythrocytapheresis and required no patient maintenance, admission to the ICU or repeated sedation as needed for femoral catheter placement. Important factors in starting a chronic pheresis program for these complex patients include the following:

1. communication between all involved departments,

2. well equipped location to support cardiac or neurologic complications

3. reliable intravenous access,

4. staff familiar with the Vortex port and pheresis catheters,

5. supportive apheresis team from the American Red Cross,

6. disclosure of possible cost to the institution and

7. standardized policies and procedures for all locations.