A Practical Approach To Apheresis In Sickle Cell

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Use of exchange transfusion in SCD.

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A Practical Approach To Apheresis In Sickle Cell

  1. 1. Overview of Apheresisand Sickle Cell Disease Raúl H. Morales-Borges, MD Medical Director American Red Cross Blood Services Puerto Rico Region
  2. 2. What is Sickle Cell Disease?• Incidence• Pathophysiology• Treatment• Apheresis Considerations – Patient History – Amount and Type of blood – Adverse Reactions
  3. 3. Incidence 30 Years Ago - Infectious Disease - Stroke - Average Life Span Today - Infectious Disease - Multiple Organs - Average Life Span
  4. 4. Peripheral Blood Smear Sickle Cells
  5. 5. Illustrationof theformationof sicklecells From Raj, A. Sickle Cell Disease in Pediatrics. Emedicine.com/ped/topic2096.htm
  6. 6. OrgansAffectedby SCD
  7. 7. Hereditary Genealogy of SCD
  8. 8. How does SCD cause problems?• Increased blood viscosity – Limits blood flow – Promotes red cell sickling – Promotes cell-cell interactions – Activation of coagulation systems• Increased cardiac output• Expanded plasma volume• Will likely have a lower hemaglobin/hematocrit
  9. 9. Sickled cellsand theireffect onblood flow from
  10. 10. Velocity = pi (pressure) (radius of tube)4 8 (length of tube) (viscosity) Classic Poiseuille equation for laminar flow of a fluid through a straight tube
  11. 11. LungsDeoxygenated RBCs Oxygenated RBCs Peripheral circulation
  12. 12. What does this mean to my patient?• By doubling the viscosity, the velocity is cut in half.• Flow is slower in vessels of small radius. – arterioles, venules, capillaries• Flow is slower in vessels with lower pressures. – Venules• Vaso-occlusive events and Red Cell Destruction
  13. 13. Treatment Options• Prophylactic vaccines and antibiotics• Hydroxyurea• Aspirin• Iron Chelation Therapy• Simple Transfusions• Red Cell Exchange
  14. 14. How do these issues impact my patient?• Acute – Vaso-occlusive stroke – Acute chest syndrome – Multiorgan failure syndrome – Right upper quadrant syndrome – Surgery/pregnancy• Chronic – Primary and secondary stroke prevention – Multi-organ problems
  15. 15. What are the potential complications of SCD that may be treated by apheresis?• Acute infarctive stroke• Acute chest syndrome• Multi-organ failure syndrome• Right upper quadrant syndrome• ? Priapism• ? Acute pain episode• ? Pregnancy
  16. 16. How does a RBC exchange transfusion help my patient?• Removing sickle cells and replacing them with normal red cells will…. – Improve the oxygen-carrying capacity of the blood – Decrease blood viscosity – Reduce vaso-occlusive events – Reduce hemolytic complications
  17. 17. Sickled cellsand theireffect onblood flow from
  18. 18. What are your treatment options?• Simple red blood cell (RBC) transfusions• Partial RBC exchange transfusion• Automated RBC exchange transfusion – Partial exchange transfusion – Full exchange transfusion
  19. 19. Advantages & Disadvantages• Simple Transfusion • Exchange Transfusion• Sickle cells remain • Removes % of sickle cells• Increases viscosity • Maintains viscosity• Increased iron load • No increases in iron load• Volume shift • Volume shift?• Easier vascular access • May need great access• Time • Time• Transfusion complications • Transfusion complications
  20. 20. Procedural Considerations• Hydration • Extracorporeal volume• Medication • Vascular Access – Diuretics – Indwelling line or – ACE inhibitors graft• Anemia – Temporary femoral – Simple transfusion dialysis catheters – Blood prime – Ports• RBCs
  21. 21. Line Access Issues and Solutions - Peripheral access - Catheters - Ports
  22. 22. Line Access Issues
  23. 23. Antigen – Antibody Reactions Y SENSITIZATION Y Y ABO YRed Cell Y Y Y Y Rh (D, C, c, E, e) Y Y Kell (K, k) YY Y Kidd (Jka, Jkb) Y Y Y Y Y Duffy (Fya, Fyb) Y Other: S, U, V, hrb Y Antibody Y Y Y Y Y Y
  24. 24. Goal: Less than 30%• Final Hematocrit – Raise it no more than 3% (why?)• Final sickle cell % of less than 30 (why?) – Approximately 1.5 red cell volumes in an untransfused patient. – Approximately 1 red cell volume in a recently transfused patient.
  25. 25. What does this mean to my patient?• By doubling the viscosity, the velocity is cut in half.• Flow is slower in vessels of small radius. – arterioles, venules, capillaries• Flow is slower in vessels with lower pressures. – Venules• Vaso-occlusive events and Red Cell Destruction
  26. 26. How much to exchange?• Prior to the exchange, need: – Starting hematocrit – Sickle cell percentage – Weight in kg – Total red cell volume
  27. 27. Calculating the Red Cell VolumeRed cell volume = (*TBV) (hematocrit)*TBV = estimated total blood volume - adult male: 60 – 75 (70)cc per kg - adult female: 55 – 70 (65)cc per kg - infant/child: 80/70cc per kg
  28. 28. Procedural Considerations• Removal of Normal Plasma Constituents• Lowers Plasma Cations – Calcium• Alteration of Pharmacodynamics• May Alter Laboratory Test Results
  29. 29. Possible Complications• Acute transfusion reactions• Vascular access complications• Procedural complications• Delayed transfusion reactions• Other
  30. 30. Potential Apheresis Adverse Reactions• Anxiety • Transfusion Reactions – Hyperventilation – Allergic reactions – Vasovagal – ABO-incompatibility• Procedural – Bacterial sepsis – Hypocalcemia – TRALI – Hemolysis – Anaphylactic – Hypo/Hypervolemia Reactions – Mechanical • Thrombosis or Hemorrhage
  31. 31. Paresthesia Consider: Consider: Hyperventilation, Oral calcium,Pause Procedure Citrate toxicity Slow infusion rate Vasovagal Yes Consider: Consider: Oral calcium, Slow pulse Hyperventilation, Knees above head Citrate toxicity Slow infusion rate Vasovagal Yes Consider: Nausea & Consider: IV Calcium, Vomiting Citrate toxicity Cold compresses ACE inhibitor Slow infusion rate Yes Consider: Consider: Convulsions IV Calcium, Citrate Toxicity, Knees above head Vasovagal Call back-up
  32. 32. Erythema, Itching, Hives Respiratory Symptoms No Yes Mild, StopPause Procedure, Call Backup Antihistamine Steroids Severe, Resume, Stop procedure,Redose if needed ABCs Call Backup
  33. 33. Hypotension Consider… Dehydration,Pause Procedure, VasovagalCheck Vital Signs Yes Change in Hyperventilation, pulse rate Vasovagal Yes Hypovolemia, dyspnea Hyperventilation, TRALI Vasovagal Cardiogenic Shock Yes Sepsis, fever Bacterial contamination, ABO incompatibility TRALI
  34. 34. Issues of Exchange Transfusion in SCD• No clear cut guidelines for palliative chronic exchange transfusion for painful vaso-occlusive crisis in adults patients (Navaid M, Melvin T: Am J Hosp Palliat Care 2010 May; 27(3):215-218).• Good results in the use of partial exchange transfusion in the management of SCD in a perioperative setting (Jaeckel R, Thieme M, Czezlick E, Sablotzki A: Journal of Medical Case Reports 2010; 4:82).• Not effective in SCD with coexisting warm autoantibody haemolysis (Baron JM, Baron BW: Blood Transfus 2010; 8:303-306).
  35. 35. Summary• What is SCD?• What are some of the complications of SCD?• What are some of the available treatment options?• How do RBC exchange transfusion help?• What are some of the indications for treatment?
  36. 36. Communication
  37. 37. Summary• What are some of the factors to be considered prior to beginning a RBC exchange procedure?• What are the goals of a RBC exchange?• What are some of the possible complications during and following a RBC exchange?• How do we recognize and manage these complications?
  38. 38. References• Sharon, BI. Management of Congenital Hemolytic Anemias. In Rossi’s Principles of Transfusion Medicine. 4th ed. AABB Press, 2009.• Special Review Issue of the Journal of Clinical Apheresis: Clinical Applications of Therapeutic Apheresis. 2010. ASFA website. http://apheresis.org.• McLeod BC, Triulzi DJ. Therapeutic Apheresis. A Physician’s Handbook, Bethesda, MD: American Association of Blood Banks, 2005.• McLeod BC, Weinstein R, Winters, JL, Szczepiorkowski, ZM eds. Apheresis: Principles and Practice. 3nd ed. Bethesda, MD: AABB Press, 2010.• Popovsky MA, ed. Transfusion Reactions, 3nd ed. Bethesda, MD: AABB Press, 2007.• The Food and Drug Administration/Center for Biologics Evaluation and Research: Code of Federal Regulations, Title 21 – Food and Drugs. http://www.fda.gov.
  39. 39. References• Technical manual, 16th ed. Bethesda, MD: American Association of Blood Banks, 2008.• Standards for blood banks and transfusion services, 26-27th ed. Bethesda, MD: American Association of Blood Banks, 2010-11.• Swerdlow P. Red Cell Exchange. Hematology. 2006:48-53. http://asheducationbook.hematologylibrary.org/cgi/content/ful l/2006/1/48• Lottenberg R & Hassell K. An evidence-based approach to the treatment of adults with sickle cell disease. Hematology. 2005: 58- 65.• Gladwin M & Kato G. Sickle cell disease: Advances in pathogenesis and management. Hematology. 2005:51-57.• Immunohematology, American Red Cross.2006:22:101-148.
  40. 40. Dr. Raúl H. Morales Borges Hematólogo/Oncólogo• Cruz Roja Americana de • Ashford Medical Center PR – Suite # 107 – Servicios de Sangre – Condado, San Juan – Ubicados en el Centro – Tel. 787-722-0412 Medico de PR – Fax 787-723-0554 – Tel. 787-759-8100 – ihoa@coqui.net – Ext. 3873 – www.ihoapr.com – Cel. 787-505-5814 – MoralesBorgesR@usa.redcross.org

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