Canterbury DHB

Potential New Case of Haemophilia

Newborn males

1. Take blood from umbilical cord (or peripheral vein) for factor level.
   • Avoid heel pricks for coagulation studies or factor assays.
   • Oral vitamin K prophylaxis is effective in preventing classical Haemorrhagic Disease of the Newborn, but ineffective in preventing late HDN. Increasing the dose or giving it weekly for a longer period increases the efficacy of the oral prophylaxis. Alternatively, IM vitamin K can be given, especially after factor replacement administered, providing pressure is maintained for a minimum of 5 to 10 minutes.
2. If factor assay indicates severe (< 1%) or moderate (1 to 4%) factor VIII/IX deficiency it is essential that the results are sensitively communicated to the parents by experienced staff.
3. Confirm diagnosis with a further factor level.
4. Follow newborn closely for a minimum of 7 days after birth through daily phone contact from Haemophilia Centre or GP and frequent midwife visits. Educate parents regarding symptoms of ICH (e.g. poor feeding, irritability, listlessness, full fontanelle, convulsions, pallor).

   Recommended imaging:

   • If clinical suspicion of ICH, perform a CT scan (in preference to ultrasound) of head.
   • Perform a CT head scan in all high-risk deliveries.
   • The need for a CT scan in all other cases depends on the clinical circumstances. Discuss all cases with a specialist centre.
5. If confirmed ICH, treat according to National Guidelines for intracranial haemorrhage.

Newborn females

Suggest factor VIII/IX level is measured on females born to carrier mothers to detect the occasional carrier female with low levels at risk of symptomatic bleeding.

Newborns with prolonged APTT but no family history

Assay factor VIII/IX levels in newborns without a family history of haemophilia if coagulation screen shows prolonged APTT, particularly:

• if significant haemorrhage occurs "spontaneously" e.g. subgaleal haemorrhage, large cephalohaematoma, unusual pattern of bruising
• if excessive bleeding occurs with procedures e.g. venepuncture, vitamin K injection, or circumcision. Do not be dissuaded from considering haemophilia if haemorrhage is a presenting feature of a newborn’s illness, even if:
  • coagulation screen suggests DIC
  • thrombocytopenia coexists with prolonged APTT

Topic Code: 5274