Bleeding Disorders inPregnancy – – Long journey of gyn&obs

GTG 71 2017

Discussed about

Haemophilia
VWD

Haemophilia – X linked Recessive

Definition
Haemophilia is an X-linked condition associated with reduction or absence of clotting factor

VIII (haemophilia A) or
IX (haemophilia B), causing bleeding symptoms.

Inheritance

50% of neonatal males with severe haemophilia have no previous family history. In these cases there is a 90% chance that the mother is a carrier, with risk to the next male child.

Also there are different phenotypes

Risks

Mother
Female carriers may have low factor VIII/IX levels
Carriers of haemophilia are at increased risk of bleeding with

invasive procedures,
termination,
spontaneous miscarriage and
at the time of delivery

Baby

Male neonates with haemophilia are at increased risk of bleeding, including

intracranial haemorrhage (ICH) and
extracranial haemorrhage (ECH)
Iatrogenic bleeding

Pre-pregnancy

baseline factor level
attention to weight and correction of any iron deficiency
Genetic counselling
following delivery

Prenatal diagnosis

preimplantation genetic diagnosis
All carriers of severe haemophilia should be offered fetal sex determination by free fetal DNA analysis from 9 weeks of gesta tion
PND with chorionic villus sampling at 11–14 weeks of gestation
Third trimester amniocentesis if diagnostic investigations have not previously been performed

Antenatal management

multidisciplinary team
factor VIII/IX should be checked at
○ booking,
○ before any antenatal procedure and
○ In the third trimester;
○ (factor VIII levels rise in pregnancy, but factor IX tends to remain stable)
VIII/IX levels of at least 0.5 iu/ml
Tranexamic acid should be considered in combination with treatment
Desmopressin (DDAVP) can be used antenatally to raise factor VIII levels.
Due to the antidiuretic effect, fluids should be restricted to 1 litre for 24 hours after use or, if not possible, electrolytes should be monitored
Recombinant factor VIII should be used if levels obtained with DDAVP are insufficient
Recombinant factor IX is required to cover invasive or surgical procedures in women with factor levels less than 0.5 iu/ml.
Have a clear plan at 37 weeks
External cephalic version should be avoided in affected or potentially affected male fetuses and female fetuses who are obligate or possible carriers of severe haemophilia B

TOD/ MOD

A decision regarding the timing and mode of delivery should be agreed jointly between the woman and the multidisciplinary team
Planned lower segment caesarean section should be discussed for the delivery of affected male babies, especially those with severe haemophilia and/or if the fetal status is unknown.

If elective caesarean section is intended, this should be at 39 completed weeks with a clear plan for the event of spontaneous labour occurring beforehand.
For intended vaginal delivery, spontaneous labour is preferred, if no other obstetric concerns, to minimise the risk of inter vention
The use of ventouse and midcavity forceps should be avoided for male babies at risk of haemophilia.
Fetal blood sampling (FBS) and fetal scalp electrode (FSE) should be avoided in babies expected to have severe or moderate haemophilia

There is no evidence to recommend special measures for delivery in women carrying female fetuses.
A female fetus who is at risk of carrying severe haemophilia B may theoretically be more at risk of ICH/ECH and this should be considered in the birth plan

Analgesia/anaesthesia

Intramuscular injections should generally be avoided.
If clotting factor levels are less than 0.5 iu/ml, DDAVP should be given to raise factor VIII, or factor IX concentrate should be given to raise factor IX, aiming for 1.0 iu/ml.

Postpartum

active management of the third stage of labour.
Levels of factor VIII/IX should be maintained above 0.5 iu/ml for at least 3 days following an uncomplicated vaginal delivery or 5 days following instrumental delivery or caesarean section.
Tranexamic acid should be continued postpartum until lochia is minimal.
Neonate
Cord blood sampling and diagnostic testing is recommended for all male babies born to mothers who are carriers of haemophilia.
A/B although some mild cases may require retesting at 3–6 months of age
Cord blood sampling and diagnostic testing of female babies born to mothers who are carriers of haemophilia is not recommende d.
In a neonate with low factor levels, vitamin K should be administered by an oral regimen
Cranial ultrasound (US) should be considered prior to discharge in all neonates with severe or moderate haemophilia.

VWD – AD / AR / Variable Penetrance

VWD is classified according to whether the deficiency of von Willebrand factor (VWF) is partial quantitative (type 1),

qualitative(type 2) or

severe quantitative (type 3)

⚫ VWD is autosomal and variably dominant or recessive, depending on the VWD type.
⚫ variable penetrance and expression – Genetic counselling should be provided for all women with VWD.
⚫ increased risk of antepartum, primary and secondary PPH.
⚫ Risk of bleeding increased in type I VWD whose VWF level does not rise above 0.5 iu/ml by term, or type 2 or 3 VWD.
For fetuses at risk of having type 2 or 3 VWD, FBS, external cephalic version, fetal scalp monitoring, ventouse delivery and midcavity forceps should be avoided.

Pre-conceptional

⚫ type I VWD whose VWF level does not rise above 0.5 iu/ml by term, or type 2 or 3 VWD.
⚫ multidisciplinary approach.

Antenatal

VWF antigen levels and activity, and factor VIII levels checked at booking, in the third trimester and prior to any invasive procedures

Clinicians should aim for factor VIII and VWF ristocetin cofactor (VWF:RCo) activity levels of 0.5 iu/ml or above to cover surgical procedures or spontaneous miscarriage.

⚫ should receive haemostatic support in the form of DDAVP, where responsive, or VWF-containing concentrates
DDAVP in preference to blood-derived factor concentrates where possible. This is safe for use in pregnancy and at delivery, but should be avoided in pre-eclampsia

Postpartum

Women with VWD should be considered for tranexamic acid for the postpartum period. A standard dose is 1 g three to four times a day for 7–14 days.

In some cases, prolonged administration for 2–3 weeks or more may be necessary.

A plan for the diagnostic testing of the neonate following delivery, including cord blood sampling, should be included in the maternal
pregnancy management plan.

⚫ Cord blood samples for VWF activity should be taken for babies at risk of type 2 and 3 VWD
⚫ Can give Vitamin K if at risk