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///2018 Abstract Details
2018 Abstract Details2019-08-02T15:57:01-05:00

Pregnant Hemophilia A Carrier and Neuraxial Analgesia

Abstract Number: T2D-8
Abstract Type: Case Report/Case Series

Ryan M. Militana D.O.1 ; Christopher Chiang M.D.2; Heather C. Nixon M.D.3

Introduction: Hemophilia A is an x-linked recessive disorder that results in a deficiency of factor VIII causing a tendency for increased bleeding. Pregnant hemophilia A carriers present a unique set of challenges to healthcare providers. An increase in coagulation factors can precipitate a normalization of coagulation; however, hemostasis may not be adequate and may increase risk of post-partum hemorrhage and neuraxial hematoma. There is limited data on the management of hemophilia carriers and neuraxial analgesia. Our case outlines a multidisciplinary approach to optimizing the patient’s care.

Case: A 19 y/o G1P0 at 37+3 with a PMH of Hemophilia A carrier, Tietz syndrome, vascular malformation presented for IOL secondary to oligohydramnios. In the first trimester, the patient consulted a genetics counselor due to a strong family history of Hemophilia and was diagnosed as a Hemophilia A carrier. History included prolonged post-surgical bleeding and menorrhagia, but denied any hemarthrosis, ecchymosis, or petechiae. Patient’s factor VIII assay level was obtained during 1st trimester and was 40 IU/dL (normal 89-180). Von Willebrand disease studies were within normal limits.

At admission, a multidisciplinary approach was employed with a hematology consult. An updated factor VIII assay was sent (59 IU/dL). No further intervention was required prior to delivery since the factor VIII levels were above 50 IU/dL. Coagulation studies and a TEG were also obtained on admission and were unremarkable. If, however, the patient had increased bleeding during or after delivery, recombinant factor VIII would be given at 50IU per kg and dosed q12h.

Although offered, the patient opted to not have any neuraxial analgesia and delivery was uncomplicated with an EBL of 600cc. Three weeks post-delivery, she noted persistent lochia with blood that required use of a pad, but no other signs of bleeding. Repeat Factor VIII assay was 59 IU/dL.

Discussion: Pregnancy is a pro-thrombotic state and is accompanied by various hemostatic changes that may modify the bleeding risks of patients with inherited bleeding disorders. Particularly when mild, pregnant patients with bleeding diathesis may develop normal clotting factor levels because of a significant pregnancy-induced rise in levels of FVIII and vWF antigen activity. Many experts suggest getting factor VIII levels to > 50% before neuraxial anesthesia. It is therefore important to check factor levels during pregnancy and particularly in the third trimester to develop an appropriate management plan for labor and delivery including neuraxial placement, bleeding prophylaxis and pharmacological interventions should PPH arise. Timed induction with Factor VIII assay and TEG may facilitate care of these patients and safety of neuraxial procedures. Factor levels that may have normalized during pregnancy tend to return to baseline within 1-3 weeks following delivery, but may drop earlier and should be closely monitored.

SOAP 2018