Abstract

Introduction

There are about 1.1 million Jehovah's witnesses (JW) in the United States who do not accept blood transfusions due to religious beliefs. When these patients present with severe anemia, management is challenging and mortality high. Hemoglobin based Oxygen Carrier HBOC-201 has been available for compassionate use, but its use is limited due to lack of experience. Here we present the experience of a tertiary care center with HBOC-201 to better manage critically ill JW patients with anemia.

Case1

59 y.o. JW female was admitted with diabetic ketoacidosis, right lower extremity gangrene, and sepsis. Labs showed WBC 17.4, Hgb 7.9, and platelets 434. Within hours, the patient went into septic shock requiring vasopressors and mechanical ventilation, AKI requiring dialysis, and transaminitis. Her Hgb dropped to 6.9. She was started on insulin drip and broad-spectrum antibiotics. Her clinical condition improved, vasopressors were discontinued and she was extubated. She subsequently had a below knee amputation. On post-operative day 2, her condition worsened. She developed fevers and vasopressors were restarted. Her Hgb dropped to 5.8. Erythropoietin injections and Iron supplementation were initiated. At this point, the surgical team planned for an above knee amputation for better control of the infection, with a pre-operative goal Hgb of 8. On post-op day 7, the patient's clinical status deteriorated, and she was re-intubated and found to have an embolic stroke. Her Hgb continued to drop to 5.3 and she subsequently received 2 units of HBOC in preparation for urgent surgical intervention for infection control. Her Hgb improved to 6.5 following administration of 2 units of HBOC. Despite the improvement in Hgb, she acutely worsened with increased vasopressor and ventilation requirement, severe acidosis, methemoglobinemia, elevated troponin (0.5), and hypoglycemia. On the next day, she went into cardiac arrest and died.

Case2

49 y.o. JW male was admitted with acute pancreatitis. Labs showed WBC 12.9, Hgb 18.6, transaminitis and lipase >600. The patient's clinical status improved with conservative management but on day 3 developed hematemesis with a drop in Hgb to 9.7. He became lethargic and was subsequently intubated. Esophagogastroduodenoscopy (EGD) showed a gastroesophageal junction clot. Gastrointestinal bleeding persisted over the next 3 days with drop in Hgb to 5. EGD showed an ulcer with visible vessel. Apart from proton pump inhibitor drip, he was also started on Erythropoeitin , B12 and Iron Despite these measures, his Hgb continued to drop with a nadir of 3.3 and worsening clinical status requiring multiple vasopressors. On Day 9, HBOC was approved for compassionate use. The patient was infused 4 units of HBOC. Hgb improved to 5.2, and the patient was weaned off vasopressors. A repeat EGD with injection and cauterization of the bleeding vessel were performed. The patient received one unit of HBOC daily to keep Hgb>5 (figure 1) and the gastroduodenal artery was embolized. The patient developed complications from HBOC, including hypernatremia, alkalosis and methemoglobinemia successfully treated with ascorbic acid and methylene blue. Troponin was normal and EKG was normal. Two days later, he developed lactic acidosis, ventilator associated pneumonia, acute respiratory distress syndrome, shock and multiorgan failure and succumbed to his illness. During this time, his Hgb was stable at 6.1 and there was no further bleeding.

Conclusion

HBOC-021 (Hemopure√Ę) is a polymerized bovine hemoglobin. It has been used for management of anemia in JW patients under compassionate-use FDA guidelines with varying success. Our experience with HBOC shows that it can be used successfully in emergencies to hemodynamically stabilize patients. Our first patient showed an improvement in Hgb while our second patient attained a stable Hgb and recovered from his hemorrhagic shock. With clinical improvement, he could get an EGD and embolization done which controlled his bleeding. The side effects of HBOC are easily managed with supportive care and methylene blue. We believe that Intensivists and Hematologists should be aggressively involved in JW patients who urgently need but refuse blood transfusion. An institution based protocol should be available to rapidly obtain HBOC. Efforts should be made to minimize blood loss and the use of erythropoiesis stimulating agents should be maximized.

Disclosures

No relevant conflicts of interest to declare.

Author notes

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Asterisk with author names denotes non-ASH members.