Management of Profound Anemia in the Setting of Blood Refusal

1. Management of Profound Anemia in the Setting of Blood Refusal Sharon Sledge, MA, MS, RN Coordinator, Patient Blood Management Hackensack University Medical Center Hackensack, New Jersey SABM Annual Meeting September 19-21, 2013 Los Angeles, California

2. Conflict of Interest DisclosureSharon Sledge, MA, MS, RN Has no real or apparent conflicts of interest to report.

3. Objectives Discuss various reasons for blood refusal Recognize the need for tolerance of anemia Identify blood conservative modalities- strategies to manage profound anemia without the use of allogeneic blood

4. Anemia-Definition • Anemia –comes from the Greek word “Anaimia”–meaning “without blood” • A decrease in the number of red blood cells, hemoglobin, or hematocrit OR • A decrease in the oxygen carrying capacity of the blood

5. WHO Definition of Anemia

11. 2 Major Reasons for Blood Refusal • Growing knowledge of risks of allogeneic blood • Faith-based avoidance of blood transfusion

12. Transfusion Risks and Blood Safety Public perception is that blood transfusion is not entirely safe. Most people would prefer not to be transfused

13. Transmittable Infections Prion (Creutzfeldt Jakob) Parvovirus B 19 Salmonellosis Syphilis Toxoplasmosis Tryponosomiasis (Chagas’ disease) TT virus Typhoid Typhus Yersinia Types, sub-types, mutant and divergent strains of the above The next virus • Babesiosis • Bartonella • Borna Disease Virus • Borrelia • Brucellosis • Cytomegalovirus • Epstein Barr • Filariasis • Hepatitis • HIV • HTLV • Human Herpesvirus 8 • Leishmaniasis • Malaria

14. Jehovah’s Witnesses • Profound respect for life • Avoid habits/activities that could cause harm such as smoking, ETOH abuse, dangerous sports, etc. • Refuse to take life • warfare • abortions

15. Why don’t JWs accept blood transfusions? • Gen 9:4 God allowed Noah and his family to add animal flesh to their diet after the Flood but commanded them not to eat the blood. God told Noah: “Only flesh with its soul—its blood—you must not eat.” This command applies to all mankind from that time on because all are descendants of Noah. • Lev 17:14 “You must not eat the blood of any sort of flesh, because the soul of every sort of flesh is its blood. Anyone eating it will be cut off.” God viewed the soul, or life, as being in the blood and belonging to him. Although this law was given only to the nation of Israel, it shows how seriously God viewed the law against eating blood. • Acts 15:20 “Abstain . . . from blood.” God gave Christians the same command that he had given to Noah. History shows that early Christians refused to consume whole blood or even to use it for medical reasons. http://www.jw.org/en/jehovahs-witnesses/faq/jehovahs-witnesses-why-no-blood-transfusions

16. Why don’t JWs accept blood transfusions? • Religious vs Medical • Biblical command to abstain from blood • God views blood as representing life • Obedience to God and respect for him as the Giver of Life

17. Patient Self Determination Act

18. Advance Directives

19. Caution “It may be tempting to engage a patient refusing blood in debate. However, this casts the clinician into an adversarial role … An alternate approach involves serving as the patient's advocate, with the focus on finding the best possible therapy within the boundaries of the patient's religious beliefs and comfort zone (ie, values and preferences).” Crookston, K.P. (2013). The approach to the patient who refuses transfusion. In A. Silvergleid, S. Kleinman, J. Tirnauer Ed.),UpToDate. Available from http://www.uptodateonline.com

20. Tolerance of Anemia

21. Tolerance of Anemia “Human cardiovascular and metabolic response to acute, severe isovolemic anemia.” JAMA Landmark study by Weiskopf, et al in 1999. Before and after interventional study. Bled normal volunteers to hemoglobin of 5g/dL Conclusion: no effect on systemic oxygenation

22. Tolerance of Anemia • Is it the patient or the physician who cannot tolerate anemia? A prospective analysis of 1854 non-transfused coronary artery surgery patients. • Senay, et al reported that for transfused and non-transfused patients: “Ventilation time, duration of intensive care unit stay, ICU and hospital re-admission, reoperation for bleeding, or tamponade, low cardiac output, postoperative new atrial fibrillation, stroke, creatinine level at hospital discharge, new onset renal failure, mediastinitis, postoperative pulmonary complication and mortality rates were similar in both groups.” Senay, et al, (2009) Perfusion, (24)6, 373-380.

23. Tolerance of anemia – HUMC Experience • Post partum hemorrhage 4.6 g/dL • Multiple trauma 5.1 g/dL • Uterine Fibroids 2.9 g/dL • These patients survived without transfusions

24. Compensatory Mechanisms of Anemia ↑Cardiac Output ↓ Viscosity Redistribution of blood flow ↑ O2Extraction

25. Support Physiologic Toleration of Anemia – Fluids Volume Therapy to Achieve Normovolemia Circulate remaining red cells Decreases viscosity of blood (assisting with increased cardiac output) Avoid volume overload which leads to further hemodilution and associated side effects (coagulopathies, tissue edema, etc.) Colloids may be needed to keep capillary beds open with extreme anemia (Hgb 4-5 g/dl)

26. Judicious Volume Replacement • Permissive Hypotension • Balanced vs. Unbalanced Solutions • Hyperchloremic metabolic acidosis • Dilutional Coagulopathy • Preservation of Microcirculation in Severe Hemodilution/Anemia • Colloids vs. crystalloids

27. Support Physiologic Toleration of Anemia – ↑ O2 Delivery • Increase FiO2 to increase O2 dissolved in plasma • Optimise ventilation/Intubation • Up to 35% of VO2 can be contributed by dissolved O2 in plasma - when hgb at 5 g/dl or less* • Hyperbaric oxygen • Role of pH, CO2 and temperature on oxygen delivery and utilization

28. Support Physiologic Toleration of Anemia – ↓ O2 Demand • Treatment of pain • Sedation • Intubation, ventilation, and paralysing agents • Antioxidants • Normothermia • Treatment/Prevention of infections • Beta-blockade

29. Optimize Hematopoiesis • Iron • PO vs. IV • Vitamin C • Erythropoiesis stimulating agents • Epoetinalfa (Procrit, Epogen) contains albumin, pre-surgical indication, may produce hemoglobin increase sooner than with Darbepoetinalfa* • Darbepoetinalfa (Aranesp) • Avoid bone marrow depressing agents Steensma, D. P., & Loprinzi, C. L. (2006). Epoetin Alfa and Darbepoetin Alfa Go Head to Head. Journal of Clinical Oncology, 24(15), 2233-2236. DOI: 10.1200/JCO.200D6.o0w5.7n7l9o4aded

30. VTE Prophylaxis • Critically low hgb does not rule out VTE risk • Erythropoiesis stimulating agents carry risk of thrombosis • VTE Prophylaxis - risk vs. benefit • Pharmacological vs. Mechanical

31. Basic PBM Principle • If the test result will not change your plan of care, don’t order the test! • Understand tolerance of anemia

32. Still True Today “Use of pedi-vials in ICU reduced total volume of blood drawn by 46.8% when compared to adult-sized collection tubes, and amount of blood collected was sufficient to perform the test in all cases.” Smoller BR et al: Reducing adult phlebotomy blood loss with the use of pediatric-sized blood collection tubes. Am J. Clinical Pathol, 1989; 91:701-703.

33. MicrosamplingTechniques • Collect laboratory specimen in pediatric tubes utilizing only 1/10th the normal amount • Microtainers-”automated friendly” • Minimize amount of labs drawn daily • Draw bloods while maintaining a closed circuit and reinfuse blood

34. Shaffer, 2007 • Use point-of-care testing with microanalyzers rather than traditional laboratory tests when available. • Refrain from drawing extra tubes of blood “in case” additional tests are needed. • Evaluate at least daily the need for routine or repetitive testing. • Use noninvasive methods whenever possible (eg, pulse oximetry rather than arterial blood gas analysis, Hgb monitors). • Schedule blood tests at the same time so that a single sample of blood can be used for multiple tests. • Consult with the laboratory about storing blood samples for later use

35. Blood-loss Flow Sheet Reduces diagnostic blood loss Raises physicians’ awareness of total volume of blood drawn in each patient Potentially can significantly reduce daily testing and transfusion requirements

36. Promptly Stop Bleeding(Less Invasive Approaches) • Angiography • Diagnose bleed • Stop bleeding/embolization – (GI, trauma, tumor) • Endoscopy • Injection Therapy • Cautery • Clips • Pharmacologic

37. Promptly Stop Bleeding Sometimes Damage Control Surgery is the Best Option • Emergent Surgical Intervention for Life Threatening Illness or Injury • Stop Hemorrhage, Control Fecal Contamination • To ICU & Re-establish Normovolemia, Acid-Base Balance, & Temperature • Return to O.R. for Second Look & Definitive Repair or Repeat Damage Control

38. Hemostasis - Systemic Bleeding • Tranexamic Acid (TXA, TA, can be applied locally) • Aminocaproic Acid (EACA, can be applied locally) • Desmopressin (DDAVP) • IV Vitamin K • Conjugated Estrogens • rFVIIa • Vasoconstrictors • Prothrombin Complex Concentrates • Cryoprecipitate

39. Individual Patient Decision • Minor Blood Fractions • Clotting Factors – includes surgical glues, cryoprecipitate, and prothrombin complex concentrates • Albumin • Epoetinalfa (Procrit, Epogen): contains albumin • Immunogobulins • Hemoglobin-Based Oxygen Carriers (HBOC) • Hemin

40. Individual Patient Decision • Patient’s Own Blood • Cell Salvage • Wound/Surgical Site Salvage • Cardiopulmonary Bypass • Dialysis • Red Cell Labeling or Tagging Studies • Acute NormovolemicHemodilution (ANH) • Platelet Sequestration • Reintroduction of Line Waste (ex. Safeset, Vamp)

41. Case Study #1 • 60 y.o. Jehovah’s Witness • No Cardiac History • Colonoscopy 1 Day Prior • Presented to ED at 0500 • Frank bloody stools at home & frequently in ED/ICU • HR 106-129, SBP 71-117, DBP 78/53 • H&H 12 & 36.6 (0855 H&H 7.3 & 22)

42. Case Study #1 • Colonoscopy less than 4 hours from arrival • Surgery & Angiography on standby if needed • Bleeding stopped with clips & injection therapy • Nadir H&H 4.0 & 11.4 • Supplemental O2 • Epo & Iron • Minimize Lab Draws • Discharged from ICU after 6 days • Home after 10 days H&H 6.0 & 18.9

43. Case Study #2 • 41 y/o African American Jehovah’s Witness • Transferred from nearby hospital with hemoglobin 2.7 g/dL • Walking to bathroom unassisted! • Diagnosis: leiomyoma • Treated with IV iron, epo • Discharged day 3 hgb 4.8 g/dL

44. Case Study #2 • Treated for 3 months with Lupron • Anemia was corrected • Hgb 11.9 day of surgery • TAH • EBL 250cc • Post op course unremarkable • Discharged PO day 2 with hgb 10.9

45. Case Study #3 “A Young Adult Jehovah's Witness With Severe Anemia” Nnenna Ukachi, Wynne Morrison, Samantha VanHorn, Revathy Sundaram and John D. Lantos Pediatrics 2013;132;547; originally published online August 19, 2013; DOI: 10.1542/peds.2013-0503 http://pediatrics.aappublications.org/content/132/3/547/full.html

46. Points to Remember • Patient’s declining transfusion may or may not find acceptable: • the use of minor blood fractions or • certain procedures involving use of the patient’s own blood • The patient declining transfusion must be consulted prior to the use of these types of interventions.

47. Take Home Message Profound Anemia + Blood Refusal Don’t Always = DEATH

48. Next time…What will you do ?

49. References • Crum, E., & Valenti, J. (2007). Can a bloodless surgery program work in the trauma setting? Nursing2007, 37(3), 54-56. • Lorentzen K., Kjær B., & Jørgensen J. (2013). Supportive treatment of severe anaemia in a Jehovah's Witness with severe trauma. Blood Transfusfusin, 19, 1-2. doi: 10.2450/2013.0263-12. [Epub ahead of print] • Seeber, P. & Shander, A. (2013). Basics of Blood Management (2nd edition.). Oxford, UK: Wiley & Sons, Ltd. • Shander, A., Javidroozi, M., Perelman, S., Puzio, T., & Lobel, G. (2012). From Bloodless Surgery to Patient Blood Management. Mount Sinai Journal of Medicine, 79:56–65. DOI:10.1002/msj.21290