Intravenous immunoglobulins in the management of acute chest syndrome in two Jehovah's Witnesses with sickle cell disease

Sickle cell disease (SCD) is an invalidating hereditary red cell disorder distributed worldwide. It is characterized by the synthesis of the pathological hemoglobin S (HbS) and its main clinical manifestations are chronic hemolysis and acute vaso-occlusive crises (VOC). These crises are the main cause of hospitalization for young adults with SCD. Up to now the standard of care for acute VOC has been based on intravenous fluid hydration, analgesia, and red cell transfusion as either simple transfusion or manual/ automatized exchange. Acute VOC in special circumstances, such as in Jehovah’s Witnesses with SCD, is one of the most challenging experiences that hematologists/internal medicine doctors and Emergency Department physicians might encounter. Jehovah’s Witnesses refuse blood transfusion on religious grounds, but they might accept fractions of plasma or blood substitutes. Indeed, recent changes in the policy of the Jehovah’s Witness community have led to plasma components, such as immunoglobulins, being considered acceptable.1,2 Since blood transfusion is a cornerstone in the treatment of acute severe sickle cell-related events, alternative therapeutic strategies might be adopted to manage acute SCD-related events before they become life-threatening conditions. Up to now, the treatment of severe VOC in Jehovah’s Witnesses with SCD has been based mainly on supportive measures (e.g., erythropoietin, folate supplementation, hypothermia, pharmacological coma) and/ or infusion of blood substitutes (e.g., hemoglobin-based oxygen carriers such as HBOC-201).3