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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]