The Obstetric Experience of Carriers of Haemophilia

Abstract

Objective To review the obstetric problems, pregnancy outcome and management of carriers of Design Retrospective review of haemophilia and maternity unit records.Setting Haemophilia Comprehensive Care Centre.Participants Thirty-two carriers of haemophilia (24 haemophilia A, eight haemophilia B) who had their obstetric care at the Royal Free Hospital over a 10-year period (1 985-1995).Main outcome measures Uptake and results of prenatal diagnosis, changes in factor levels during pregnancy, effect of knowledge of fetal gender on obstetric management and neonatal outcome, and maternal haemorrhagic complications.Results There were 82 pregnancies and 32 resulted in miscarriage or social abortion. The option of prenatal diagnosis was taken up in only 35% (17/48) of pregnancies. There were five affected male fetuses diagnosed prenatally but only three women opted for termination of the pregnancy. Knowledge of fetal gender was unavailable to the attending obstetrician in 46% (21/46) of pregnancies. A fetal scalp electrode was applied in eight, fetal blood sampling was performed in four, and ventouse delivery was conducted in one of these pregnancies. No adverse effects were reported from the first two procedures, but the ventouse delivery was associated with a huge cephalhaematoma requiring blood transfusion. On the other hand, in five cases fetal blood sampling was withheld because fetal gender was unknown. Four of the eight caesarean sections performed might have been avoided if the gender had been known. The incidence of primary and secondary postpartum haemorrhage was high, 22% (including two cases with massive haemorrhage) and 11 %, respectively.Conclusion Carriers of haemophilia A and B require special obstetric care with close liaison with the haemophilia centre, and management guidelines should be available and observed. Knowledge of fetal gender is very valuable for management in labour and should be determined antenatally even if the mother declines prenatal diagnosis.haemophilia.

The obstetric and gynaecological management of women with inherited bleeding disorders – review with guidelines produced by a taskforce of UK Haemophilia Centre Doctors’ Organization

“…Factor VIII levels have been shown to increase significantly in carriers of haemophilia A during pregnancy. Although the majority of patients will develop levels within the normal range, the rise is variable, and a small proportion may still have low levels at term [27,28]. In contrast, FIX levels do not rise significantly in carriers of haemophilia B [28].…”

Management of Inherited Bleeding Disorders in Pregnancy

“…60,61 One case series reported the use of FBS in an affected male baby with no adverse consequences. 52 There were no identified case reports of bleeding secondary to the use of FSE in fetal bleeding disorders.…”

“…52 Those with low levels in the third trimester can have higher risk even following factor concentrate. 72 Carriers should be identified as being at increased risk of PPH and offered uterotonics for the third stage of labour.…”

How I treat von Willebrand disease

“…The risk of bleeding is very small when FVIII:C and VWF:RCo levels are more than or equal to 50 IU/dL, which also is the minimum level that should be achieved for a safe spinal or epidural anesthesia. 66,67 In type 1 VWD pregnant women with FVIII:C levels lower than 50 IU/dL, desmopressin on the day of parturition and for a couple of days thereafter is advisable. To prevent late bleeding, VWF:RCo and FVIII:C levels should be tested, and the women should be monitored clinically for at least 2 weeks postpartum.…”