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?
