Sickle cell crisis in third trimester of pregnancy complicated by hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome

Abstract

Introduction: Sickle cell disease is one of the most common hemoglobinopathies in the world, affecting approximately 100,000 people annually in the United States alone. This case presents an example of the importance of close monitoring during the pregnancies of patients with sickle cell disease in order to facilitate prompt evaluation and treatment of complications as they arise. Case Report: A 22-year-old, gravida 2 para 1001, was admitted at 306/7 weeks of gestation due to sickle cell crisis. Following admission and observation, she developed hemolysis, elevated liver enzymes, and low platelets (HELLP) syndrome on hospital day-4, and decision was made to proceed with delivery. The patient was maintained on magnesium seizure prophylaxis for 24 hours following delivery. The patient was discharged home on post-operative day-5 on labetalol 400 mg q8hrs secondary to persistent hypertension. The patient was doing well at her routine six week postpartum visit and maintained on the anti-hypertensive medication. Conclusion: This case of HELLP syndrome following a sickle cell crisis highlights the importance of close monitoring of sickle cell disease patients during pregnancy. Multi-disciplinary care plays a critical role in the successful management of these patients due to the acute decompensation that can follow a sickle cell pain episode. A facility must be prepared to provide support from obstetrics, hematology, neurology, and intensive care. The obstetrical team must understand sickle cell physiology. The resources to provide prompt blood transfusion and exchange transfusion must also be available. This knowledge is especially important to health care providers who may be taking care of patients in lower resource settings.