Variation in symptoms between hemophilia A carriers during labor and after delivery can lead to different clinical presentations requiring individualized management

There needs to be increased awareness about the challenges Hemophilia carriers A can potentially experience during labor and after delivery

NHF 2017 (Chicago, IL August 24-26): Management of women who are hemophilia carriers during labor and after delivery (called the post-partum period) is not yet standardized. Most of these carriers have normal pregnancies without any bleeding. Levels of Factor VIII usually increase significantly in pregnancy ,unlike levels of factor IX, which do not usually change significantly. This means carriers of hemophilia A might be less likely to have problems. After delivery, a carrier’s factor level drops down to pre-pregnancy levels, which increases the chance of bleeding, called post-partum hemorrhage or PPH. In some parts of the world, PPH remains a major cause of death in mothers who are carriers of hemophilia.

Results from the poster, Management of Hemophilia Carriers around the Time of Their Delivery: Phenotypic Variation Requiring Customization of Management were presented at the Annual Meeting of the National Hemophilia Foundation (NHF) and describe three case reports that demonstrate how symptom variation between patients can lead to different clinical presentations to healthcare professionals requiring customization of management. Two of the three case reports described clinical outcomes for symptomatic hemophilia A carriers and the third report described an asymptomatic hemophilia A carrier.

The first patient is a 34 year old symptomatic hemophilia A carrier with baseline Factor VIII of 20% (mild hemophilia) that increased to 61% (within the normal range of 50-150%) in the third trimester. She went into labor in her 34th week of pregnancy. She received a recombinant FVIII an hour prior to epidural anesthesia, and 1 more dose 1-2 hours prior to delivery. She gave birth to a girl through a vaginal delivery complicated with post-partum hemorrhage due to lack of contractions of the uterus (called atony) and part of the placenta remaining in the uterus (called a retained placenta). She needed to have a dilation and curettage (D&C) in the OR to remove it. Bleeding improved with massaging the uterus and administering medication to make the uterus contract, after which she received another dose of rFVIII. Following delivery, she was treated with rFVIII for 2 days with factor levels of 94% and 75% and was treated after going home for 6 weeks with FVIII.

The second patient was the 31 year old sister of the first women, an asymptomatic hemophilia A carrier with a normal baseline level of 133% (within normal range of 50-150%) that increased to 212% in the third trimester. As she was known to be carrying a boy, so  forceps, vacuum or fetal scalp electrodes were avoided. She had an epidural injection with no complications, without any need for factor infusion, and underwent an uncomplicated vaginal delivery at full term (40 weeks) of an unaffected boy. Her factor level was 152% the day after giving birth.

The third patient is a 34 year old symptomatic hemophilia A carrier with baseline FVIII level of 46% (below normal range of 50-150%, meeting CDC classification of mild hemophilia [<50%]) that increased to 96% in the third trimester. She received recombinant FVIII for the first delivery (a boy with severe hemophilia A) and had heavy bloody discharge for 6 weeks after delivering. She is currently at full term with her second baby, and plans are to treat her through delivery and for several weeks with FVIII.

It is essential to close the gap in the care of hemophilia carrier women by raising awareness of the challenges they may face and managed in a collaborative multidisciplinary approach.