von Willebrand disease

Question 1

Discuss preconceptual counselling for a woman with vWD including plan for care during pregnancy

Answer 1

• Autosomal dominant condition with 50% chance of transmitting variant to offspring.
  
  • A person who inherits an abnormal vWF gene from both parents has type 3 vWD (considered to be autosomal recessive; very severe disease).
• Invasive prenatal testing only indicated for families with type 3 vWD.
• Risks to mum:
  
  • Generally no issues as vWF levels increase during the 2nd and 3rd trimesters.
  • Bleeding more likely to occur postpartum.

Antenatal:

• MDT care: haematologist, MFM
• Avoid aspirin
• Create plan for management of bleeding or if procedures are needed:
  
  • TXA
  • vWF concentrate
  • DDAVP
• vWF and factor VIII activity levels, platelet count: 2nd trimester, 3rd trimester (ideally 34/40).
• Anaesthetic review: regional anaesthesia
  
  • vWF activity level ≥50 adequate for regional.

Intrapartum:

• Deliver in hospital
• IVL, FBC, G&H.
• Check and maintain vWF and factor VIII levels at 50 or higher with concentrates.
• Regional anaesthesia: consult anaesthesia.
• CS reserved for normal indications.
• Management of bleeding:
  
  • DDAVP after labour started or near delivery time. Repeat doses Q12H max 4 doses.
    
    • Risk of hyponatremia and fluid overload: fluid restriction and check serum sodium
  • vWF concentrate
  • TXA

Postpartum:

• Avoid invasive procedures on neonate until vWD status established.