Introduction: Hemoglobin SC sickle cell disease in the setting of pregnancy with pre-eclampsia or HELLP syndrome (Hemolysis, Elevated Liver enzymes, Low Platelet count) is a challenging clinical scenario that greatly increases maternal and fetal risks during pregnancy. Hemoglobin SC sickle cell disease is a variant of sickle cell disease characterized by the presence of both hemoglobin S and C. This can exacerbate pregnancy complications through its vaso-occlusive properties, which can impair placental function, lead to fetal growth restriction, and increase the risk of pre-eclampsia, preterm birth, stillbirth, and maternal morbidity and mortality. Pre-eclampsia is a hypertensive disorder of pregnancy paired with proteinuria that occurs after 20 weeks gestational age. It can lead to HELLP syndrome, a severe form of pre-eclampsia characterized by hemolysis, elevated liver enzymes, and thrombocytopenia. The pathophysiology of HELLP syndrome involves endothelial dysfunction and microangiopathic hemolytic anemia, which can be particularly severe in the setting of Hemoglobin SC sickle cell disease due to an underlying hemolytic state. Management of Hemoglobin SC sickle cell disease in the setting of pregnancy with pre-eclampsia or HELLP syndrome requires a multidisciplinary approach, involving OB/GYNs, hematologists, and sometimes even gastroenterologists, to optimize maternal and fetal outcomes and provide patient-centered care. Methods: Case report. Case Description: Patient is a 27-year-old G1 with a history of Hemoglobin SC sickle cell disease (managed with hydrocodone outpatient), asthma, retinopathy, and avascular hip necrosis. She was admitted at 37+0 weeks for a sickle cell pain crisis characterized by 10/10 lower back pain with radiation to her bilateral hips and legs. Her pain was unrelieved with hydromorphone, and she declined increases to her medications due to her concern for fetal harm after her biophysical profile (BPP) showed a score of 6/10- likely secondary to the effects of narcotics. She ruled into pre-eclampsia without severe features based on proteinuria and mildly elevated blood pressures. As a result, she was induced at 37+3 weeks with a spontaneous vaginal delivery complicated by postpartum hemorrhage (PPH) of 882 mL. After delivery, she developed tachycardia of 147. Her hemoglobin dropped from 10.7 g/dL to 8.6 g/dL a few hours prior to delivery, then to 5.6 g/dL at 4 hours after delivery. Her alanine aminotransferase (ALT) was 444 units/L and her aspartate aminotransferase (AST) was 865 units/L. She experienced reactive leukocytosis with up trending white blood cell count of 25.37 x 103/mcL, but was asymptomatic with negative urine and blood cultures. She was started on cefepime, vancomycin, and metronidazole for prophylaxis. Her platelets had been down trending throughout her hospitalization. Her thrombocytopenia in combination with pre-eclampsia led to the diagnosis of pre-eclampsia with severe features. A coagulation panel was obtained and trended after the PPH, and it showed worsening levels with lactate dehydrogenase of 990 units/L, an international normalized ratio increase to 1.37, and a fibrinogen drop from 316 to 280 mg/dL. Due to her anemia, she was transfused with 2 units of packed red blood cells (pRBCs). Her tachycardia and sickle cell pain continued despite escalating hydromorphone doses, and there was concern for HELLP syndrome versus acute worsening of sickle cell crisis. Due to her hemodynamic instability and worsening of her labs, she was transferred to the MICU. An electrocardiogram showed sinus tachycardia. Splenic ultrasound showed stable splenomegaly. A computed tomography angiogram (CTA) was negative for acute pulmonary embolism but showed bilateral small pleural effusions with atelectasis. Her right lower extremity doppler ultrasound was negative. In the MICU, she was treated with magnesium for seizure prophylaxis, and she remained stable without seizure activity. Her pain regimen was escalated per sickle cell protocols, and her pain improved. She received an additional 2 units of pRBCs for hemoglobin of 6.4 g/dL, and her hemoglobin stabilized at 9.9 g/dL. Additionally, her platelets stabilized at 146x103/mcL, and her ALT and AST down trended. Additional imaging showed grade 1 hepatic steatosis. A CT brain without contrast was unremarkable. Once she was stabilized, she continued to meet postpartum milestones and remained hemodynamically stable. She also continued to deny symptoms of pre-eclampsia. Her reactive leukocytosis improved, and antibiotics were discontinued. Discussion: In the case of this patient, several discussions and many multidisciplinary decisions were made to optimize maternal and fetal outcomes and manage postpartum complications with the goal of minimizing long term negative effects and organ damage to the mother. Hydromorphone can be a mainstay of treatment for sickle cell pain crises, but in the setting of pregnancy, some women may be reluctant to take the medication due to potential risks of poor fetal growth, stillbirth, preterm delivery, neonatal abstinence syndrome, and the need for cesarean delivery. In this patient’s case, her BPP score was 6/10, suspected to be secondary to hydromorphone use. Due to patient concern about this, the increased doses were held prior to delivery, and other management routes were taken, which demonstrated patient-centered care.
Torrance et al. (Sat,) studied this question.