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Table 2 Summary of management with haemophilic preterm patient

From: Severe haemophilia A in a preterm girl with Turner syndrome: case report – a diagnostic and therapeutic challenge for a paediatrician (Part 2)

Labour and delivery o Vaginal delivery or caesarean section
o Minimalise risk of bleeding/haemorrhage- avoid prolonged labour
o No vaccum
o No forceps
o No fetal scalp elecrodes
o No fetal blood samping
o Post-delivery screening of VIII clotting factor (umbilical cord)
o No invasive procedures till results.
[3, 33]
Ventilation o non-invasive and mechanical ventilation (all modes) if required
o focus on nose care
[29, 34]
Central venous catheter o factor replacement prior to procedure [3]
Pawilizumab o intramuscular injection
o factor replacement prior to vaccination
o application of ice to the injection site
o compression at the injection site
[31]
Vaccination o vaccinations (mandatory and recommended) according to institutional vaccination schedule
o subcutaneous administration preferred
o application of ice to the injection site
o thinnest possible needle
[31]
Pain o minimalisation of invasive procedures
o cumulation of prcedures
o no nonsteroidal anti-inflammatory drugs (NSAIDs) or acetylsalicylic acid (ASA)
o usage of acetaminofen, morphine
o tramadol, codeine, ketamine, fentanyl
[3]
Surgical procedures o pre-operative inhibitor (anti- FVIII antibodies) screening
o factor replacement therapy prior to procedure (or in case of presence of FVIII antibodies-therapy with by-passing coagulation agents)
o reservation of VIII coagulation factor or by-passing agents for the surgery and postoperative time, rehabilitation
o re-screen level of anti- FVIII antibodies after surgery
o surgical procedures at or in consultation with a comprehensive hemophilia treatment centre.
[3]
PDA ligation o usage of paracetamol,
o avoid ibuprofen
[3, 29, 34]