Management of Inherited Bleeding Disorders in Pregnancy Flashcards
(23 cards)
Which clotting factor is reduced/absent in Haemophilia A?
Factor VIII (8)
Which clotting factor is reduced/absent in Haemophilia N?
Factor IX (9)
What is the hereditary pattern for Haemophilia?
X-linked
How is cell free fetal DNA used for carriers of severe haemophilia?
To determine the sex of the baby - All carriers of severe haemophilia should be offered fetal sex determination by free fetal DNA
analysis from 9 weeks of gestation
How would haemophilia status of a male fetus be determined in pregnancy?
prenatal diagnosis with CVS at 11-14/40 or third trimester amniocentesis
In pregnancy, what happens to factor VIII and IX levels normally?
Factor VIII rises, factor IX tends to remain stable
What is the aim for factor VIII/IX levels to cover surgical/incasive procedures or miscarriage?
Aim for levels of at least 0.5 iu/mL
What can be used antenatally to raise factor VIII levels?
Desmopressin (DDAVP); fluids should be restricted to 1L for 24h after use, monitor electrolytes; recombinant factor VIII should be used if levels obtained with DDAVP are insufficient or in a known non responder
What can be used antenatally to raise factor IX levels?
Recombinant factor IX - is required to cover invasive or surgical procedures in women with
factor levels less than 0.5 iu/ml
What is type 1/2/3 von Willebrand Disease?
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).
What should be tested in women with VWD ?
VWF antigen levels and activity, factor VIII levels checked at booking, in the third trimester and prior to any invasive procedures
What should the aim for VWF:RCo activity levels be to cover for surgical procedures or spontaneous miscarriage?
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.
Clinicians should be aware that patients with type 2B VWD may develop ________
following DDAVP treatment.
thrombocytopenia
When can central neuraxial anaesthesia be given to patients with type 2 VWD?
Should be avoided unless VWF activity is >0.5 iu/mL; factor VIII level is >0.5 iu/ml and the haemostatic defect has been corrected.
Can NSAIDS be given?
Intramuscular injections and postnatal nonsteroidal anti-inflammatory drugs (NSAIDs) are only
suitable in the short term when VWF activity and factor VIII are more than 0.5 iu/ml.
How is factor X1 deficiency inherited?
It is an uncommon autosomal disorder which has both recessive and dominant inheritance patterns
What is the incidence of Factor Xi deficiency in non Jewish population?
1 in 1 million
What are the therapeutic options for Factor Xi deficiency?
TXA, factor Xi concentrate, FFP;
TXA and Factor XI should not be given simulaltaneously- increases thrombotic risk
Can central neuraxial anaesthesia be given to Factor Xi deficient patients?
Not to women with low factor XI levels with a known bleeding phenotype/unclear phenotype
How are most rare inherited bleeding disorders inherited?
autosomal recessive- eg factors II, V, VII, X and XIII, and combined factor V+VIII deficiencies?
What is another name for clotting Factor II?
Prothrombin
When would you give prothrombin concentrate in Prothrombin deficiency?
If factor II activity is <0.2 iu/mL and significant bleeding, established labour or prior to C section; 20-40iu/kg at 48h intervals to achieve factor II activity 0.2-0.4 iu/mL
