An inherited bleeding disorder, haemophilia is caused by a mutation in one of the genes that is responsible for blood clotting which is located on the “X” chromosome. Women who are haemophilia carriers may have mild bleeding symptoms due to reduced clotting factor levels in their blood.

Most carriers have normal pregnancies without any bleeding complications. However, clotting factor levels should be tested in the third trimester when they are at their highest. Normal levels of the clotting factors are between 50%-100%. If the levels are low (less than 50%), precautions should be taken during labour to reduce the risk of excessive bleeding. Factor VIII levels increases during pregnancy and reduces the risk of bleeding in haemophilia A carriers, while factor IX levels remain low throughout pregnancy, therefore haemophilia B carriers are at risk of excessive bleeding.

Precautions for healthcare professionals

- Avoid using plasma-derived factor concentrates, as they may transmit parvovirus B19 (Fifth disease that may cause the baby to develop severe anaemia and the mother to have miscarriage).

- If tests have shown that a woman is likely to suffer from bleeding during or after delivery, preventive treatments should be given such as anti-fibrinolytics (tranexamic acid, aminocaproic acid) and von Willebrand factor concentrates.

- It should be assumed, unless prenatal testing has shown otherwise, that the foetus is also affected by a bleeding disorder. Thus, delivery should be as gentle as possible for both the woman and the baby. Natural delivery without the use of instruments is the goal here.

- In order to prevent bleeding, the following should be avoided whenever possible:
o suction extraction of the baby
o epidural
o deep intramuscular injections
o unnecessary episiotomy
o use of forceps
o scalp electrodes
o caesarean section (unless the woman has received factor concentrates)

Postpartum care

After delivery, a carrier’s circulating clotting factor returns to the low pre-pregnancy levels, which in turn increases the risk for postpartum haemorrhage (PPH). 

Postpartum haemorrhage (PPH) is a major cause of maternal death and disability, therefore the carriers need extra care after delivery.

Certain precautions can be taken to reduce the risk of PPH such as oxytocin to keep the womb contracted, and the placenta should be delivered by controlled cord traction of the umbilical cord. MIMS

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Sources:
https://www.cdc.gov/ncbddd/haemophilia/women.html
https://haemophilianewstoday.com/women-with-haemophilia__trashed/pregnancy-and-haemophilia/
http://www.hsm.org.my/