Treating a Patient With Your Hands Tied: Acute Chest Syndrome in a Jehovah’s Witness

Abstract

SESSION TITLE: Student/Resident Case Report Poster - Critical Care I SESSION TYPE: Student/Resident Case Report Poster PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM INTRODUCTION: Acute chest syndrome (ACS) in patients with sickle cell anemia (SCA) is treated with simple or exchange transfusion, analgesics, antibiotics and IV fluids. We present a Jehovah’s Witness (JW) patient with ACS whose religious beliefs precluded use of blood products. She was treated successfully with supportive care (including mechanical ventilation, sedation, paralysis, erythropoesis stimulation) and survived despite concomitant hemolytic and aplastic crisis and a hemoglobin (Hb) nadir of 3.1gm/dl. CASE PRESENTATION: A 26 year-old Black female JW with SCA presented with sickle pain crisis affecting the torso and extremities. Examination revealed pallor, icterus, tachycardia with clear lungs and abdominal tenderness. Lab data: Hb 7.7gm/dl, WBC 32, platelets 52; Reticulocyte count 2%; HbS 55%; LDH 6077 U/L; haptoglobin 24mg/dl; total bilirubin 2.8 mg/dl. Patient was treated with analgesics, IV fluids, oxygen (O2) therapy and empiric antibiotics. In 48 hours, Hb dropped to 5.2 gm/dl; oxygenation worsened with diffuse lung infiltrates on chest x-ray [CXR] (Figure 1). She was transferred to the ICU for management of ACS. Patient refused transfusion of blood products, with the awareness that death might occur as a consequence. She subsequently required mechanical ventilation for respiratory distress. To minimize O2 consumption (VO2) and energy expenditure, patient was sedated and paralyzed. To increase O2-delivery by increasing O2-solubilty in plasma, 100% FiO2 was used. This was titrated down after 3 days to avoid O2-toxicity. Patient also received erythropoietin, IV iron, folate, thiamine, vitamin B12, vitamin C, antibiotics, IV fluids. Blood draws were limited with Pedi-tubes used when necessary. Table 1 shows Hb trend. Lowest Hb was 3.1gm/dl. Paralytics were discontinued after a week. After 2 weeks, blood counts and CXR began to improve; patient was extubated at 4 weeks. At discharge, a week later, Hb was 7.7g/dl. DISCUSSION: Difficulty in caring for JW patients with ACS stems from inability to deliver 1st-line therapy of blood transfusion due to religious convictions. Hence, physicians should be aware of alternative options available in their management. Therapeutic measures reported to be successful in management of ACS in JW patients include: hyperbaric O2, barbiturate coma, hypothermia and blood substitutes. Polymerized Hb products and synthetic O2-carrying products have been successfully used, but remain investigational, are not FDA-approved or readily accessible. In this case, supportive therapy was successful in treating ACS without blood transfusion. CONCLUSIONS: Supportive measures such as mechanical ventilation, deep sedation and paralysis to decrease VO2, erythropoesis stimulation and minimizing blood loss can be successful in treating JW patients with ACS whose beliefs may pose barriers to the mainstays of treatment. Reference #1: Remmers PA et al. Clinical strategies in the medical care of Jehovah’s Witnesses. Am J Med 2009; 119:1013-18. DISCLOSURE: The following authors have nothing to disclose: Deepak Vadhera, Frank Senatore, Debapriya Datta No Product/Research Disclosure Information