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Case Study 1: Red Cell Exchange

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Case Study 1: Red Cell Exchange Case Study 1: Red Cell Exchange Education Session VI: Pediatric Apheresis Leon Su, MD Section Chief, Transfusion Medicine and Apheresis Disclosures • None Patient HM • 8 year old female SCD • History of CVA in 2012 • Hgb S levels well maintained on chronic tx (20-30%) • Ferritins and Iron in liver (MRI quantification) elevated despite chelation therapy • Began chronic exchange therapy in early 2015 When to consider outpatient red cell exchange (Sickle cell disease, non-acute) • Stroke prophylaxis/iron overload prevention • ASFA Category II, Recommendation 1C • Vaso-occlusive pain crises • ASFA Category III, Recommendation 2C • Pre-Op management • ASFA Category III, Recommendation 2A Exchange Transfusion Simple Transfusion Reduced transfusional iron Iron overload more common Unrestricted by baseline HCT Restricted to lower baseline with ability to target end HCT HCTs to avoid high viscosity Multiple donor exposures Less donor exposure Schwartz et al. J. Clin Apheresis 28:145-284, 2013 Kim et al. Blood 83(4):1136-1142, 1994 Access Options • Peripheral access – 18 gauge dialysis-type steel needles – 18 to 20 gauge angiocaths • AV fistula and grafts • Central venous catheters – Tunneled and Nontunneled • Ports (indicated for power injection) – Single and bilateral – Dual lumen Double lumen ports • 9.5 Bard powerport: 40-50 mL/min • 11.4 Angiodynamics dual lumen smart port: 50-60 mL/min • Considerations when choosing port – Previous history of clots and infections – History of peripheral access – Evaluation by apheresis nurses – Appropriate size based on patient Preparing a child for port access: child life support resources “They looked into my mouth and into my ears; they looked into my eyes and touched my tummy. But, they never looked at me.” (7 year old patient) What is a Child Life Specialist? • Assist children in • Assessment managing/understanding • Play: therapeutic play, medical healthcare experiences play, developmental play • Preparation • Coping strategies • Pre-admission tours • Educate • End of Life/ Bereavement work • Sibling support • Back to school • Supervise Child Life Support Initial access Ready for exchange Blood Product Selection • HM is DcE/DcE with antibody to little e. • Requires C, K, Fy(a), Jk(b), S and little e negative blood Institutional Guidelines • Type and screen 1-3 days prior to exchange • Rh and Kell matched if negative screen • Also Fy, Jk and S matched if positive screen • Hgb S negative, no irradiation unless other indication • < 14 days old • Communication with blood bank and blood provider paramount to ensure availability of blood Prime versus nonprime • Extracorporeal volume (ECV) • Blood warmer volume (BWV) • 10-15% of TBV should not exceed ECV + BWV • If not priming, intraprocedure HCT can be calculated • Optia – Prime with saline/albumin during RBCX results in a mix of replacement fluid (RBCs) with saline/albumin in the return line to a HCT = patient Monitoring Hgb S and procedure parameters • Pre and post Hgb S levels • Target pre procedure Hgb S levels below 30% • HM fluctuates between 10-30% pre and post procedure • Frequency start at every 4 weeks • Typical FCR 40% • AC infusion rate 1.1 with 15 mg/kg/hr Calcium Gluconate or AC infusion rate 0.8 with no calcium • WB:AC ratio 15:1 Ferritin Iron (mg) 900 250 800 700 200 600 150 500 400 100 300 200 50 100 0 0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 40 • Procedure 14 and 15 had 35 frequency of 5 weeks before 30 procedure 25 • Depletion performed with procedures 4, 12 and 13 20 • Minimizing iron gain 15 Pre HCT – Lower end target HCT 10 – Combining with depletion Post HCT 5 • End target HCT – what’s the right target? 0 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 Adverse reactions • HM tolerates procedures well and has not had any adverse events over her 1.5 year course • Premedicated with Tylenol and Benadryl • Other patients with hypotensive reactions and allergic reactions – Managed with corticosteroids and fluid boluses, one patient with washed red cells Summary • HM continues to have good Hgb S control at 4 week interval RBCX • Her Ferritins came down from the 1500s to the 400-600s with exchange and better compliance with iron chelation therapy. • Plan is to keep at 4 week intervals which will hopefully help pre procedure HCTs stay >24% and allow for modest depletion with exchanges • May require splenectomy down the road Thank you!.
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