Birth and post-partum
At the time of birth the median maternal age was 32 years, haemoglobin 125g/L and platelet count 218x10^9/L. Neuraxial analgesia was administered in 52 (40%) births, advised against in 9 (7%) and refused in 4 (3%) despite documented adequate haemostatic potential. Normal vaginal birth occurred in 62% (81) of births with intervention required in 12% including forceps (9%), scalp electrodes (1%) and vacuum extraction (1%). Intervention was required in 2 births of women with X linked conditions where the sex of the fetus was unknown and in 1 woman with a more severe form of VWD; only 1 infant was subsequently found to be affected and there were no documented complications. Caesarean sections were performed in 38% (49) of births with the decision directed by maternal choice or obstetric indications. Of these 14% were emergency caesarean sections (Table 1).
Factor replacement was administered in 4 pregnancies as prophylaxis at the time of obstetric intervention or treatment of pregnancy-related bleeding complications. Replacement factor or platelets were required at the time of 37 births. There were 2 women who received factor despite normalisation of levels in pregnancy, this included a women with Type 1 VWD and Mild HA. In both cases this was to facilitate out of hours neuraxial analgesia when urgent repeat factors levels were not available. Post-partum factor replacement was not continued for these women. In addition 3 women with hypofibrinogenaemia only received replacement at the time of birth otherwise all other women who received factor at the time of birth had it continued in the post-partum period. Two women with Type 2B VWD did not receive factor at the time of birth despite inadequate third trimester levels however factor was commenced immediately post-partum, one of these births was complicated by a PPH. There were two further women who only received factor replacement post-partum for bleeding complications despite adequate factor levels. This included a woman with Type 1 VWD who developed a wound haematoma at the caesarean section site in a previous pregnancy and a woman with Type 1 VWD who developed a perineal haematoma. Only 1 woman who received platelets at the time of birth for an inherited platelet disorder required ongoing platelet support post-partum.
Only 36% of women received antifibrinolytics in the post-partum period. Women with more severe bleeding disorders, who required factor at the time of birth were more likely to be prescribed antifibrinolytics with 68% of these women prescribed tranexamic acid compared to 24% of women who did not receive factor at the time of birth. There were no documented cases of venous thromboembolism
PPH complicated 29 (22%) births with 48% of these associated with a major PPH as defined by an estimated blood loss greater than 1000ml. Most PPHs (26, 90%) were primary and occurred in a range of IBD as highlighted in Figure 2 2. 12 (46%) women had achieved levels above those recommended for safe birth, 13 (50%) women received planned replacement factor or platelet transfusion at the time of birth and 1 (4%) woman with Type 2B VWD did not receive factor replacement despite inadequate factor levels. An obstetric cause was identified in 12 (46%) cases of primary PPH and included retained products of conception, vaginal wall trauma, uterine atony, placental abruption and abnormal placentation or uterine anatomy. The IBD was thought be solely responsible in 2 (8%) haemorrhages and the cause was not apparent or available in 35% of cases. Secondary PPH occurred in 2 women in 3 births with 1 lady with Type 2B VWD experiencing 2 secondary PPH, one was a massive PPH; an obstetric cause was found in 2 (67%) instances (See Appendix 1).