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.

Patterns of recurrence of postpartum hemorrhage in a large population-based cohort

“…12, 22–24 Other congenital coagulation disorders, including Factor II, VII, X, XI deficiencies and carriage of hemophilia A and B are rare, but have also been associated with excess bleeding with delivery. 12, 24–26 While based on small studies, the prevalence of undiagnosed coagulation disorders in the general population of patients with PPH is low, 27, 28 and further study is needed to ascertain whether this may not pertain to the population of women that experience recurrent PPH.…”

Obstetric Management of Adolescents with Bleeding Disorders

“…33 Because women generally continue to bleed after clotting factors have returned to pre-pregnancy levels, women with bleeding disorders may be particularly vulnerable to delayed or secondary postpartum hemorrhage during this time. While delayed or secondary postpartum hemorrhage is rare, occurring after fewer than 1% of deliveries, 34,35 delayed postpartum hemorrhage has been reported in 20e25% of women with VWD, 36,37 2e11% of hemophilia carriers, 38,39 and 24% of women with factor XI deficiency. 37 …”

Gynaecological and obstetric management of women with inherited bleeding disorders

“…This risk is clearly increased to 16% to 22% in patients with vWD, with factor XI deficiency, and for hemophilia carriers [42,47]. The risk of late postpartum hemorrhage is also increased to 11% to 24% in women with bleeding disorders compared to less than 1% in the general population [41,42,47]. It is generally recommended to keep factor levels above 0.5 U/ml for 3 to 4 days after a vaginal delivery and 4 to 5 days after a Caesarean section [45].…”