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Labor Analgesia in a Patient with Paroxysmal Nocturnal Hemoglobinuria Requiring Intrapartum Anticoagulation
Abstract Number: F-38
Abstract Type: Case Report/Case Series
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disorder characterized by complement-mediated RBC destruction. Patients with PNH have an increased risk of thrombosis as well as hemolytic crises during periods of stress(1). Anesthesiologists must balance the benefit of stress reduction with the risk of hematoma when using neuraxial anesthesia in the setting of anticoagulation. Reports of successful anesthetic management of laboring patients with PNH are important in establishing a safety record for this population.
A 37 year-old G1P0 with a 9 year history of PNH presented to L&D with PROM at 38 weeks gestation. Given her risk of thrombosis, the patient was started on SQ fondaparinux at 6 weeks and switched to SQ heparin at 36 weeks in anticipation of delivery. The care team planned for intrapartum heparin infusion, to be discontinued 4 hours before anticipated delivery. During antepartum anesthesia consultation, the patient expressed a desire for epidural analgesia and exhibited clear understanding of the risk of neuraxial hematoma. Her last dose of heparin was 14 hours preadmission. aPTT was initially elevated; upon normalization an epidural catheter was placed atraumatically at the L2-L3 interspace. Following negative test dose, 10mL of 0.125% bupivacaine was given, and a 12mL/h infusion of 0.083% bupivacaine with 2mcg/mL fentanyl was started. A heparin infusion was initiated 2 hours later at a fixed rate based on the patient’s preadmission requirement. Serial neurologic exams by nursing and anesthesia staff revealed no concern for excessive motor blockade. Approximately 9 hours after discontinuation of heparin, a healthy infant was born. Upon confirming normal aPTT, the epidural catheter was removed with complete resolution of the block. A heparin infusion was restarted 6 hours after delivery. Delivery EBL was 300mL. The patient was ultimately transitioned to enoxaparin and discharged without complication on postpartum day 2. She was very pleased with her labor analgesia.
Maternal and fetal mortality rates in PNH are exceptionally high (11% & 7%)(2). A major cause of mortality is thrombosis, thus peripartum anticoagulation is common(1). If neuraxial anesthesia is offered, coordination between care teams is paramount. Factors to consider when determining candidacy for neuraxial anesthesia include the patient’s desire for labor analgesia, dosing of anticoagulation, blood dyscrasias, and anticipated difficulty of intubation should a CD be required. Large-scale studies in this population are impractical; therefore, case reports are critical to our developing knowledge base.
1)Stocche RM et al. Labor analgesia in a patient with paroxysmal nocturnal hemoglobinuria with thrombocytopenia. Reg Anes Pain Med 2001;26:79-82.
2)Ray JG et al. Paroxysmal nocturnal hemoglobinuria and the risk of venous thrombosis: review and recommendations for management of the pregnant and nonpregnant patient. Haemostasis 2000;30:1017