Case 83 – update 3 

Our pregnant patient had a factor VIII level of 54%. She is a carrier of intron 22 inversion as predicted from family history.  The free fetal DNA testing confirms she is carrying a male fetus. The patient has decided that she does not wish to undergo CVS for diagnosis.

She was keen for an elective c-section as she lives some way from the specialist centre and after careful discussion this was arranged for 37 weeks. A comprehensive care plan was agreed with MDT:

  • Premise is to assume baby may have Severe Haemophillia A 
  • C-section planned for 37 weeks safe to proceed if factor VIII >50% and also safe for spinal if threshold reached. To deliver between 9-5 and inform MDT on initiation of section.
  • VTE prophylaxis safe if VIII level >60%
  • Mechanical VTE prophylaxis and adequate hydration to be encouraged.
  • If admission in spontaneous labour no fetal blood has sampling or fetal scalp electrodes. No forceps or ventousse.

At delivery:

  • Urgent cord blood for factor VIII level.
  • Oral vitamin K not IM.
  • Any bleeding concerns contact Haem consultant on call.
  • Neonatal team to review on delivery.
  • Cranial USS will be required if affected baby.
  • Actively managed third stage of labour. 

She has a successful C-section and factor VIII level at delivery on the neonate is 36%

Does this exclude baby being affected by severe Haemophillia A? 

What should you tell the parents?

What are the generally expected levels of factors in neonates? 

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