Bleeding disorders and pregnancy
Genetic counselling and pre-pregnancy care § Assessment of the genetic risk § X-linked v. others § Reproductive options § Conceive naturally, have prenatal diagnosis and terminate if foetus affected § Accept risk of having an affected child § Not have a child § Adopt § Assisted conception with donor egg or sperm § Preimplantation genetic diagnosis § IVF to create embryos § Tests 1 or 2 cells from each embryo § Selects unaffected embryos for implantation § Success rates much lower than for natural conception § Prenatal testing § Invasive § CVS § 11-14 weeks – not before 10 weeks due to risk of limb defects § Better than amnio (>15 weeks) as can be performed in first trimester – when termination likely to be more acceptable § Miscarriage risk 1-2% § As amnio as later, foetal sex can be accurately determined, excluding females from the risk § Cordocentesis § Factor levels on cord blood § Rarely done, but may be useful if genetic mutation cannot be identified § Need to exclude maternal blood contamination as this will affect results § May need to give mum factor prior to the invasive procedure, as her levels are unlikely to have risen this early in pregnancy § Remember anti-D § Non-invasive § Ultrasound § In 2nd trimester and beyond, foetal sex can be accurately determined, and if female can reassure § Free foetal DNA in maternal circulation § To look for presence or absence of Y chromosome § Choices if foetus found to be affected
Antenatal management § Factor VIII levels usually rise during pregnancy § Factor IX levels don’t usually rise § Management § Levels should be checked at booking, 28 and 34 weeks. § Recombinant products should be used § Virally inactivated products still have risk of transmitting parvo and hep A, and parvo may be life threatening to the foetus § DDAVP controversial · Risks of placental insufficiency, miscarriage, preterm labour · Doesn’t pass into breast milk, therefore may be used in labour/ post-partum · Can be used in vWD, but repeated administrations / use in pre-eclampsia should be avoided. Monitor closely for water retention.
Intrapartum management § Delivery plan should be made in advance, planned at a unit with the necessary expertise
Labour management § Check factors and send group and save § If levels <50, treatment likely to be needed. § Spontaneous labour should be allowed where possible § If induced, greater risk of prolonged labour or instrumentation and elective section should be considered § Analgesia § Should be discussed with anaesthetist in advance § If coag screen normal and levels >50, regional block is not contraindicated § Should be performed by an expert anaesthetist § Levels should be checked prior to removal as factor levels may fall quickly after delivery
Foetal monitoring § Foetal scalp electrodes or blood sampling should be avoided
Delivery § Vacuum extraction, forceps and prolonged labour should be avoided
Post partum § Levels fall rapidly after delivery § Increased risk of primary and secondary PPHs § Factor levels should be maintained >50 for at least 3 days or 5 days if C/S § Active management of 3rd stage and minimize perineal trauma § DDAVP and TA can be considered
Neonates § IM injections and venepuncture should be avoided § Vit K should be given orally § Immunisations should be given subcut § Circumcision should be delayed until diagnosis known § Cord blood should be checked on all male babies § Cranial USS/ CT. Consider prophylaxis.
Gynaecology
Menorrhagia § Pictorial blood assessment charts should be considered in the assessment of menorrhagia § A significant number of people with menorrhagia have an underlying bleeding disorder (usually vWD) and testing should be considered. § Remember hypothyroidism § Treatment options include § Tranexamic acid § COCs § DDAVP § POP § Mirena
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