Treating a Patient with Your Hands Tied: Acute Chest Syndrome in a Jehovah's Witness.

Deepak Vadehra,Tammy Davino,Debapriya Datta

doi:10.7759/cureus.7769

Abstract

Acute chest syndrome (ACS), a vaso-occlusive crisis in patients with sickle cell anemia, is a life-threatening condition and a leading cause of death in these patients. It is treated with analgesics, antibiotics, intravenous fluid, supplemental oxygen (or ventilatory support in severe cases) with simple or exchange transfusion, being the mainstay of therapy. We report a young Jehovah’s Witness (JW) patient with sickle cell anemia who presented with ACS. Her religious beliefs precluded the use of blood products. Despite concomitant hemolytic and aplastic crisis and a hemoglobin nadir of 3.1 g/dL, the patient was treated successfully with supportive care - including mechanical ventilation, sedation, paralysis, and erythropoiesis stimulation - and survived. A maximal supportive strategy consisting of ventilatory support with a high fraction of inspired oxygen, sedation, paralysis, erythropoiesis stimulation, and limitation of blood draws can result in the successful treatment of JW patients who refuse blood products.

Highlights

Acute chest syndrome (ACS) is a vaso-occlusive crisis of the pulmonary vasculature, occurring in patients with sickle cell anemia
Simple blood transfusions are typically reserved for ACS cases which are of moderate severity, while more severe cases warrant exchange transfusion [2]
Jehovah's Witness (JW) patients refuse to accept blood transfusions on religious grounds, which makes the treatment of such patients with severe anemia a challenge [3]

Summary

Introduction

Acute chest syndrome (ACS) is a vaso-occlusive crisis of the pulmonary vasculature, occurring in patients with sickle cell anemia. A 26-year old African-American female, who was a JW with a history of sickle cell disease (SCD; hemoglobin SC), presented to the emergency room with a pain crisis involving the back, arms, legs, and chest Her exam was normal except for pallor. Her hemoglobin (Hb) at admission was 7.7 g/dL; serum lactate dehydrogenase (LDH) was elevated at 6077 IU, serum haptoglobin was reduced at 24 mg/dl; serum bilirubin and liver enzymes were elevated She was admitted for an acute pain crisis and treated with intravenous fluids and analgesics. LDH and haptoglobin levels improved indicating resolution of hemolysis After two weeks, her Hb and Hct began to show slow but steady improvement (Figure 2), and her chest X-ray demonstrated improvement as well. Graphic depiction of the trend of the patient’s hemoglobin (Hb g%) and hematocrit (Hct %) during the hospital stay

Discussion

Conclusions

Disclosures

Jonsen AR